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Vascular: Vasculitis Imaging Pearls - Educational Tools | CT Scanning | CT Imaging | CT Scan Protocols - CTisus

Imaging Pearls ❯ Vascular ❯ Vasculitis
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  • “The diagnostic accuracy of total-body computed tomography (CT) has been well established for the identification of septic shock, allowing for a rapid and simultaneous study of multiple body areas, generating detailed and panoramic images. The aim of this article is to review the characteristics of septic shock from an imaging perspective, beyond the underlying causes and to highlight how CT can be used to identify a variety of septic shock-related signs that are collectively described as CT hypoperfusion complex. The latter describes a set of widely reported signs and symptoms that are commonly observed during trauma-associated hypovolaemic shock and can be used to identify septic shock. The early recognition, diagnosis, and treatment of septic shock have profound prognostic and therapeutic implications.”
    Computed tomography imaging of septic shock. Beyond the cause: the "CT hypoperfusion complex".  
    Di Serafino M, et al.  
    A pictorial essay. Insights Imaging. 2021 Jun 5;12(1):70. doi: 10.1186/s13244-021-01006-5. PMID: 34089401; PMCID: PMC8178660.
  • “In addition to being used to identify the underlying cause of the septic state, CT is required for the early recognition of shock-associated CT imaging signs, collectively referred to as CT hypoperfusion complex, which can improve patient prognosis and management. The CT hypoperfusion complex is frequently associated with hypotension, which can also present in many no sepsis related clinical conditions, such as trauma-induced hypotensive shock (e.g. severe head or spine injury), cardiac arrest, and diabetic ketoacidosis. The CT hypoperfusion complex has important prognostic and therapeutic implications and must be promptly recognized. However, although the pathogenic mechanisms that underlie hypotensive shock and septic shock are quite different, the CT findings associated with these two syndromes are often comparable to those that have been widely described in previous literature as in post-traumatic hypotensive shock, which can be grouped into vascular, visceral, and parenchymal signs.”
    Computed tomography imaging of septic shock. Beyond the cause: the "CT hypoperfusion complex".  
    Di Serafino M, et al.  
    A pictorial essay. Insights Imaging. 2021 Jun 5;12(1):70. doi: 10.1186/s13244-021-01006-5. PMID: 34089401; PMCID: PMC8178660.
  • “These signs include the decreased enhancement of the viscera, the increased mucosal enhancement and luminal dilation of the small bowel, the mural thickening and identification of fluid-filled loops in the small bowel, the halo sign and flattening of the inferior vena cava (IVC), reduced aortic diameter, peripancreatic oedema and other controversial parenchymal and visceral findings and ascites that can occur in varying combinations and are often and reversible during early stages. The presence of 2 or more vascular, visceral, or parenchymal signs is necessary to establish the presence of CT hypoperfusion complex.”
    Computed tomography imaging of septic shock. Beyond the cause: the "CT hypoperfusion complex".  
    Di Serafino M, et al.  
    A pictorial essay. Insights Imaging. 2021 Jun 5;12(1):70. doi: 10.1186/s13244-021-01006-5. PMID: 34089401; PMCID: PMC8178660.
  • “The CT hypoperfusion complex has important prognostic and therapeutic implications and must be promptly recognized. However, although the pathogenic mechanisms that underlie hypotensive shock and septic shock are quite different, the CT findings associated with these two syndromes are often comparable to those that have been widely described in previous literature as in post-traumatic hypotensive shock, which can be grouped into vascular, visceral, and parenchymal signs. These signs include the decreased enhancement of the viscera, the increased mucosal enhancement and luminal dilation of the small bowel, the mural thickening and identification of fluid-filled loops in the small bowel, the halo sign and flattening of the inferior vena cava (IVC), reduced aortic diameter, peripancreatic oedema and other controversial parenchymal and visceral findings and ascites that can occur in varying combinations and are often and reversible during early stages. The presence of 2 or more vascular, visceral, or parenchymal signs is necessary to establish the presence of CT hypoperfusion complex.”
    Computed tomography imaging of septic shock. Beyond the cause: the "CT hypoperfusion complex".  
    Di Serafino M, et al.  
    A pictorial essay. Insights Imaging. 2021 Jun 5;12(1):70. doi: 10.1186/s13244-021-01006-5. PMID: 34089401; PMCID: PMC8178660.
  • “Abnormal renal perfusion typically manifests as an increased and prolonged parenchymal enhancement; however, focal and heterogeneous enhancement can also be observed. A fall in systolic pressure causes intense efferent glomerular arteriolar vasoconstriction, which drives glomerular filtration, leading to tubular stasis and the increased resorption of salt and water. Renal parenchymal enhancement is dependent on several factors, including cardiac output and scans timing relative to the injection of contrast agent and, thus, is a non-specific sign. However, kidney enhancement can vary depending on the severity of systemic hypotension. In some cases, unlike hyperenhancement, the decreased enhancement of the renal medulla can be observed in the venous phase, likely due to the impairment of contrast medium outflow from the renal cortex to the medulla, induced by acute renal tubular dysfunction and associated with poor prognosis.”  
    Computed tomography imaging of septic shock. Beyond the cause: the "CT hypoperfusion complex".  
    Di Serafino M, et al.  
    A pictorial essay. Insights Imaging. 2021 Jun 5;12(1):70. doi: 10.1186/s13244-021-01006-5. PMID: 34089401; PMCID: PMC8178660.
  • “Multidetector CTA with IV contrast can be used to evaluate for the presence of at-risk atherosclerotic plaque or thrombus in the aorta in its entirety. CTA is useful in the assessment of the size, extent, and location of an embolic source in the aorta, which can aid in management decisions. Aortic intraluminal thrombus is oftentimes associated with aneurysm, particularly in the abdomen, which is readily detected by CTA . A number of small studies have used CTA to detect aortic mural thrombus that was suspected of embolization. Specific data on the sensitivity and specificity of this imaging modality are lacking.”
    ACR Appropriateness Criteria  Workup of Noncerebral Systemic Arterial Embolic Source
    Vincent G. Parenti et al.
    J Am Coll Radiol 2023;20:S285-S300.
  • “Conventional catheter aortography has largely been replaced by noninvasive imaging modalities such as CTA and MRA given their high sensitivity/specificity for detecting aortic pathologies such as mural thrombus. Aortography is typically used as an alternative diagnostic strategy following initial noninvasive imaging and when therapeutic interventions are being considered.”
    ACR Appropriateness Criteria  Workup of Noncerebral Systemic Arterial Embolic Source
    Vincent G. Parenti et al.
    J Am Coll Radiol 2023;20:S285-S300.
  • Vasculitis: Differential Dx
    - PAN
    - SAM (Segmental Arterial Mediolysis)
    - FMD
  • “Segmental arterial mediolysis (SAM) is a non-inflammatory, non-atherosclerotic vasculopathy mostly involving the abdominal arteries. SAM was recently recognized as a more prevalent aetiology of abdominal pain than initially thought by healthcare providers. It is still a commonly missed diagnosis in patients with recurrent emergency room (ER) visits for abdominal pain. Most published case reports in the past have highlighted catastrophic sequelae such as intra-abdominal haemorrhage requiring surgical intervention. We report a case of SAM where the diagnosis was initially missed. After diagnosis, conservative medical management was offered which led to clinical improvement.”
    Segmental Arterial Mediolysis: An Under-Recognized Cause of Chronic Abdominal Pain.  
    Chatterjee T, Stephens J, Roy M
    Eur J Case Rep  Intern Med. 2020 Jul
  • “Positive angiographic evidence of disease in the visceral arteries is found in 40-90% of patients with PAN at the time of presentation.Arteries of the extremities and small branches of the aorta are less commonly involved. The typical angiographic feature is the presence of micro-aneurysms. Arterial steno-occlusive lesions are less common.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa  
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227 
  • “PAN is a focal pan-mural necrotising vasculitis involving small- and medium-sized arteries with multiple organ  system involvement to varying degrees, The kidneys are the most common site of involvement (70-80%) with the renal artery most frequently involved, followed by the  gastrointestinal tract (GIT), peripheral nerves and skin (50% each) and skeletal muscles (30%).  The central nervous system is involved in 10% of cases. Rare sites of involvement are the lung, heart, testicles, and spleen.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa  
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227 
  • “In conclusion, CT, including CTA, is a comprehensive imaging method that can assist in the diagnosis of PAN in occult cases based on the combination of arterial and parenchymal abnormalities. Knowledge of various CT/CTA abnormalities may obviate the need for angiography and biopsy, which is beneficial considering the invasive nature and potential complications associated with these interventions.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa  
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227 
  • “None of the CT studies reported arterial changes in PAN  besides aneurysms; however, in the present study arterial  changes were classified similar to those reported on angi-  ography. In their review of the angiographic findings in 56  patients with PAN, Stanson et al.reported occlusive lesions  in 39% cases. The spectrum of findings included luminal  irregularity, stenosis, and occlusion. Similar changes were  found in 11 arteries in the present study; however, in the  study of Stanson et al., the SMA was the most common site  of involvement followed by the hepatic and splenic arteries.  Similarly, ectasia was not reported in CT studies of  PAN. Ectasia is less common compared to aneurysms and  stenosis and was reported in 12.5% of patients, SMA being  the most commonly involved artery.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa  
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227 
  • “Segmental arterial mediolysis is a rare nonatheroscle-rotic,  noninflammatory  disease  affecting  middle-aged  and  older  patients  (median  age,  55  years)  with  a  mild  male  predominance  (male-to-female  ratio,  1.5:1) Vacuolization and lysis of the outer media originate arte-rial gaps and patchy transmural loss of the external elastic lamina,  resulting  in  formation  of  dissecting  hematomas  and aneurysms due to weakening of the wall. The main differential  diagnoses  include  fibromuscular  dysplasia,  which  is  usually  asymptomatic  and  affects  younger  patients with predominant involvement of the renal arteries, and polyarteritis nodosa.”
    Segmental Arterial Mediolysis.
    Borde P, Vilgrain V.
    Radiology. 2022 Mar;302(3):515. 
  • “Segmental arterial mediolysis (SAM) is a rare but serious nonatherosclerotic, noninflammatory vasculopathy of unknown etiology that often results in dissection, aneurysm, occlusion, or stenosis of, primarily, the abdominal arteries. Current literature lacks consensus on diagnostic criteria and management options for SAM.”
    Segmental arterial mediolysis (SAM): Systematic review and analysis of 143 cases.  
    Skeik N, Olson SL, Hari G, Pavia ML.  
    Vasc Med. 2019 Dec;24(6):549-563. 
  • Patients with SAM were most commonly men (68%) in their 60s. Hypertension (43%), tobacco use (12%), and hyperlipidemia (12%) were common comorbidities. Abdominal pain (80%) and intraabdominal bleeding (50%) were the most common presenting symptoms. Computed tomography was the most frequently used imaging method (78%), and histology was available in 44% of cases. The most commonly affected vessels were the superior mesenteric (53%), hepatic (45%), celiac (36%), renal (26%), and splenic (25%) arteries with aneurysm (76%), dissection (61%), and arterial rupture (46%). Treatments included coil embolization (28%), abdominal organ surgery (24%), open arterial repair (21%), and medical management (20%). Case-specific treatment modalities yielded symptom relief in the vast majority (91%) of patients, with a mortality rate of 7%.
    Segmental arterial mediolysis (SAM): Systematic review and analysis of 143 cases.  
    Skeik N, Olson SL, Hari G, Pavia ML.  
    Vasc Med. 2019 Dec;24(6):549-563. 
  • “Abdominal pain (80%) and intraabdominal bleeding (50%) were the most common presenting symptoms. Computed tomography was the most frequently used imaging method (78%), and histology was available in 44% of cases. The most commonly affected vessels were the superior mesenteric (53%), hepatic (45%), celiac (36%), renal (26%), and splenic (25%) arteries with aneurysm (76%), dissection (61%), and arterial rupture (46%). Treatments included coil embolization (28%), abdominal organ surgery (24%), open arterial repair (21%), and medical management (20%). Case-specific treatment modalities yielded symptom relief in the vast majority (91%) of patients, with a mortality rate of 7%.”
    Segmental arterial mediolysis (SAM): Systematic review and analysis of 143 cases.  
    Skeik N, Olson SL, Hari G, Pavia ML.  
    Vasc Med. 2019 Dec;24(6):549-563. 
  • “SAM most commonly affects the abdominal aortic branches, such as the celiac, mesenteric, and/or renal arteries with occasional carotid, cerebral, and coronary artery involvement. Depending on the involved artery and underlying pathology (dissection with stenosis versus rupture), patients with SAM may present with chronic abdominal pain, acute mesenteric ischemia, or even hemorrhagic shock. Previous case studies have reported high rates of emergent presentation related to arterial rupture and hemorrhage in approximately two-thirds of patients, with a mortality rate reaching up to 50%.”
    Segmental arterial mediolysis (SAM): Systematic review and analysis of 143 cases.  
    Skeik N, Olson SL, Hari G, Pavia ML.  
    Vasc Med. 2019 Dec;24(6):549-563. 
  • Vasculitis: Differential Dx
    - PAN
    - SAM (Segmental Arterial Mediolysis)
    - FMD
  • “Segmental arterial mediolysis (SAM) is a non-inflammatory, non-atherosclerotic vasculopathy mostly involving the abdominal arteries. SAM was recently recognized as a more prevalent aetiology of abdominal pain than initially thought by healthcare providers. It is still a commonly missed diagnosis in patients with recurrent emergency room (ER) visits for abdominal pain. Most published case reports in the past have highlighted catastrophic sequelae such as intra-abdominal haemorrhage requiring surgical intervention. We report a case of SAM where the diagnosis was initially missed. After diagnosis, conservative medical management was offered which led to clinical improvement.”
    Segmental Arterial Mediolysis: An Under-Recognized Cause of Chronic Abdominal Pain.  
    Chatterjee T, Stephens J, Roy M
    Eur J Case Rep  Intern Med. 2020 Jul
  • SAM is a non-inflammatory, non-atherosclerotic vasculopathy of unclear aetiology, with a propensity to involve splanchnic vessels but it can also affect carotid, renal and intracranial vessels. Arterial vasospasm is thought to lead to dissection of arterial walls, although the exact pathogenesis of the disease remains unclear. nother pathophysiological mechanism suggested is vacuolar degeneration leading to disruption of the outer arterial media eventually causing aneurysms and dissections. SAM mainly affects middle-aged and elderly patients. Abdominal pain is the most common clinical presentation, while it can also present as shock due to haemorrhage, haematochezia, chest pain  or stroke.”
    Segmental Arterial Mediolysis: An Under-Recognized Cause of Chronic Abdominal Pain.  
    Chatterjee T, Stephens J, Roy M
    Eur J Case Rep  Intern Med. 2020 Jul
  • “Positive angiographic evidence of disease in the visceral arteries is found in 40-90% of patients with PAN at the time of presentation.Arteries of the extremities and small branches of the aorta are less commonly involved. The typical angiographic feature is the presence of micro-aneurysms. Arterial steno-occlusive lesions are less common.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa  
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227 
  • “PAN is a focal pan-mural necrotising vasculitis involving small- and medium-sized arteries with multiple organ  system involvement to varying degrees, The kidneys are the most common site of involvement (70-80%) with the renal artery most frequently involved, followed by the  gastrointestinal tract (GIT), peripheral nerves and skin (50% each) and skeletal muscles (30%).  The central nervous system is involved in 10% of cases. Rare sites of involvement are the lung, heart, testicles, and spleen.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa  
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227 
  • “In conclusion, CT, including CTA, is a comprehensive imaging method that can assist in the diagnosis of PAN in occult cases based on the combination of arterial and parenchymal abnormalities. Knowledge of various CT/CTA abnormalities may obviate the need for angiography and biopsy, which is beneficial considering the invasive nature and potential complications associated with these interventions.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa  
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227 
  • “None of the CT studies reported arterial changes in PAN  besides aneurysms; however, in the present study arterial  changes were classified similar to those reported on angi-  ography. In their review of the angiographic findings in 56  patients with PAN, Stanson et al.reported occlusive lesions  in 39% cases. The spectrum of findings included luminal  irregularity, stenosis, and occlusion. Similar changes were  found in 11 arteries in the present study; however, in the  study of Stanson et al., the SMA was the most common site  of involvement followed by the hepatic and splenic arteries.  Similarly, ectasia was not reported in CT studies of  PAN. Ectasia is less common compared to aneurysms and  stenosis and was reported in 12.5% of patients, SMA being  the most commonly involved artery.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa  
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227 
  • “Vasculitis is a systemic disease characterized by noninfectious inflammation of the blood vessels. The cause can be primary (idiopathic) or secondary to an underlying dis- ease. The precise pathophysiology of idiopathic vasculitides is unknown. Therefore, classification is based on vessel size—large, medium, small, and variable. Large-vessel vasculitis (LVV) predominantly involves the large vessels (aorta and its main branches) but can also affect medium or small vessels. In variable-vessel vasculitis (VVV), any vessel can be affected, and no type of vessel predominates. Secondary vasculitis, or vasculitis associated with an underlying disease or specific cause such as rheumatoid arthritis, sarcoidosis, or IgG4-related disease (IgG4-RD), can also affect large vessels.”
    Multimodality Imaging of Large-Vessel Vasculitis, From the AJR Special Series on Inflammation
    Ayaz Aghayev
    AJR 2022; 218:213–222
  • "Idiopathic LVV has two subtypes: giant cell arteritis (GCA) and Takayasu arteritis (TAK). GCA is the most common idiopathic granulomatous vasculitis and involves large and medium blood vessels in patients more than 50 years old. GCA is more frequent in women and classically affects the extracranial branches of the carotid artery. Involvement of the superficial temporal arteries is known as temporal arteritis or cranial GCA (c-GCA). Common clinical characteristics of c-GCA include headache, scalp tenderness, jaw claudication, and visual symptoms. On the basis of autopsy and imaging studies, GCA can in- volve the aorta and its branches (particularly the subclavian and carotid arteries) along with c-GCA or can occur in isolation, which is known as large-vessel GCA (LV-GCA).”
    Multimodality Imaging of Large-Vessel Vasculitis, From the AJR Special Series on Inflammation
    Ayaz Aghayev
    AJR 2022; 218:213–222
  • "TAK is also an idiopathic granulomatous LVV with female predominance, though it affects younger patients (< 40 years old). It is the most common cause of aortitis in young patients and can extend to the main aortic branches. TAK can also involve the pulmonary arteries. Compared with GCA, TAK more frequently involves the symmetric arch vessels, the abdominal aorta and its branches (renal and mesenteric arteries), and the coronary arteries. Isolated left subclavian artery involvement is also more common in TAK.”
    Multimodality Imaging of Large-Vessel Vasculitis, From the AJR Special Series on Inflammation
    Ayaz Aghayev
    AJR 2022; 218:213–222
  • "Behçet disease is a systemic disorder of unknown cause that commonly affects young men. Clinically, patients can present with oral and genital ulcers, uveitis, or clinical manifestations related to affected organ systems. Although the most common vascular pathology associated with Behçet disease is venous thrombophlebitis, 2.2–18% of patients present with arterial in- volvement. The most common arterial pathology is aneurys- mal dilation of the aorta, femoral artery, or subclavian artery. Compared with other types of vasculitides, aneurysms in Behçet disease are usually saccular or pseudoaneurysms with partial luminal thrombosis and a thickened or enhancing wall.”
    Multimodality Imaging of Large-Vessel Vasculitis, From the AJR Special Series on Inflammation
    Ayaz Aghayev
    AJR 2022; 218:213–222

  • Multimodality Imaging of Large-Vessel Vasculitis, From the AJR Special Series on Inflammation
    Ayaz Aghayev
    AJR 2022; 218:213–222
  • "FDG PET/CT also has prognostic value in patients with LVV. In one multicenter study, increased FDG uptake in the aorta was a risk factor for aortic complications, such as aneurysm or dissection. Two other studies found that intense FDG uptake along the aorta and extensive branch involvement predict less fa- vorable treatment response and relapse after treatment.  A major drawback of FDG PET/CT is that it can be affected by immunosuppression treatment, which decreases its diagnostic sensitivity for LVV. However, the diagnosis can still be obtained if performed within the first 3 days of glucocorticoid treatment. Atherosclerosis is also a confounding factor in FDG PET/CT examinations. However, FDG uptake in atherosclerosis is usually patchy and less than or similar to liver uptake. An additional major limitation of FDG PET/CT is the difficulty of obtaining insurance approval in outpatient settings.  
    Multimodality Imaging of Large-Vessel Vasculitis, From the AJR Special Series on Inflammation
    Ayaz Aghayev
    AJR 2022; 218:213–222
  • Vasculitis is a systemic disease characterized by non-infectious inflammation of the blood vessels. The cause can be primary (idiopathic) or secondary to an underlying disease. The precise pathophysiology of idiopathic vasculitides is unknown. Therefore, classification is based on vessel size—large, medium, small, and variable. Large vessel vasculitis (LVV) predominantly involves the large vessels (aorta and its main branches) but can also affect medium- or small-sized vessels. In variable-vessel vasculitis (VVV), any vessel can be affected, and no type of vessel predominates.  
    Multimodality Imaging of Large Vessel Vasculitis
    Ayaz Aghayev
    https://doi.org/10.2214/AJR.21.26150
  • Primary Vasculitides: Categories
    - large vessel vasculitis (LVV): affecting predominantly large blood vessels (aorta and its major branches)
    --- Takayasu arteritis (TA)
    --- giant cell arteritis (GCA)
    - medium vessel vasculitis (MVV): affecting predominantly medium blood vessels (main visceral arteries and their branches) 
    --- polyarteritis nodosa (PAN)
    --- Kawasaki disease (KD)
    - small vessel vasculitis (SVV): affecting predominantly small blood vessels (intraparenchymal arteries, arterioles, capillaries, and venules)
    --- antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)
    ----- microscopic polyangiitis (MPA)
    ----- eosinophilic granulomatosis with polyangiitis (EGPA) (formerly known as Churg-Strauss syndrome)
    ----- granulomatosis with polyangiitis (GPA) (formerly known as Wegener granulomatosis)
    --- immune complex SVV
    ----- anti-glomerular basement membrane (anti-GBM) disease (Goodpasture syndrome)
    ----- cryoglobulinaemic vasculitis (CV)
    ----- IgA vasculitis (IgAV) (Henoch-Schönlein purpura)
    ----- hypocomplementaemic urticarial vasculitis (HUV) (anti-C1q vasculitis)
  • Primary Vasculitides: Categories
    - variable vessel vasculitis (VVV): affecting no predominant type of vessel and can affect vessels of any size and type
    --- Cogan syndrome (CS)
    --- Behçet syndrome (BS)
    - single-organ vasculitis (SOV): affecting vessels of any size in only a single organ
    --- cutaneous leukocytoclastic angiitis
    --- cutaneous arteritis
    --- primary central nervous system vasculitis (see central nervous system vasculitides)
    --- isolated aortitis (see aortitis)
    --- others (e.g. idiopathic, pauci-immune pulmonary capillaritis)
  • Secondary vasculitides: facts
    - vasculitis associated with systemic disease
    --- lupus vasculitis
    --- rheumatoid vasculitis (see rheumatoid pulmonary vasculitis)
    --- sarcoid vasculitis
    --- others (eg. Sneddon syndrome)
    - vasculitis associated with probable etiology
    --- hepatitis C virus-associated cryoglobulinaemic vasculitis
    --- hepatitis B virus-associated vasculitis
    --- syphilis-associated aortitis
    --- drug-associated immune complex vasculitis
    --- drug-associated ANCA-associated vasculitis (e.g. propylthiouracil 2) 
    --- cancer-associated vasculitis
    --- others (e.g. varicella zoster virus-associated vasculitis)
  • Giant Cell Arteritis: Facts
    Giant cell arteritis, the most common form of vasculitis in the elderly, is characterized by granulomatous inflammation of arteries, which can lead to serious, life-threatening condi- tions including aortic aneurysms, ruptures, and dissections as well as blindness. Since GCA can be treated by immunosuppressant therapy, such as corticosteroids, early diagnosis and treatment may reduce the risk of serious disability and morbidity. While temporal artery biopsy is considered the gold standard to diagnosis giant cell arteritis, it is intrusive with inherent risks as well as unreliable due to tissue sampling. Imaging studies, such as computerized tomography, are nonintrusive and have been shown to identify vasculitis including giant cell arteritis.
    Giant cell arteritis: A case report and review of literature 
    Matthew A. Crain et al.
    Radiology Case Reports 16 (2021) 3734–3738 
  • “The diagnostic criteria per American College of Rheumatology (ACR) requires at least 3 of the following: age >50 years, new onset localized headache, temporal artery tenderness or reduced pulsation, elevated erythrocyte sedimentation rate ≥50 mm/hour, and abnormal arterial biopsy (demonstrating vasculitis with predominantly mononuclear cell infiltration, granulomatous inflammation or evidence of giant cells).”
    Thoracic imaging finding of rheumatic diseases.  
    Gul M et al.  
    J Thorac Dis. 2020;12(9):5110-5118. 
  • "Thoracic complications include arm claudication or subclavian steal syndrome if the subclavian artery is affected. Involvement of the thoracic or abdominal aorta can also cause aortic valve regurgitation, aortic aneurysm and aortic dissection. In addition, Giant cell arteritis is associated with increased risk of myocardial infarction, stroke, and peripheral vascular disease.”
    Thoracic imaging finding of rheumatic diseases.  
    Gul M et al.  
    J Thorac Dis. 2020;12(9):5110-5118. 
  • "TA is a chronic, large vessel granulomatous disease that predominantly affects the aorta and its branches, leading to luminal stenosis and aneurysms in these vessels. It can also affect the coronary and pulmonary arteries. This disease is more common in women, particularly in Asian women, and often presents in patients under the age of 40. Also known as the “pulseless disease”, clinical findings in TA may include weak or absent peripheral pulses, arterial bruits, carotidynia, angina, claudication and >10 mmHg discrepancy in blood pressure between upper limbs.”
    Thoracic imaging finding of rheumatic diseases.  
    Gul M et al.  
    J Thorac Dis. 2020;12(9):5110-5118. 
  • "The classic manifestation of TA on CT angiography is concentric mural thickening, that is several millimetres wide, of the involved arteries. Other imaging options include Positron emission tomography (PET), which in combination with CT (PET-CT) or MR (PET-MR), can be used to evaluate for possible large-vessel vasculitis. The “hot” segments of the artery (i.e., those with increased standardized uptake values) can help diagnose large vessel vasculitis.”
    Thoracic imaging finding of rheumatic diseases.  
    Gul M et al.  
    J Thorac Dis. 2020;12(9):5110-5118. 
  • “CTA can evaluate mural thickening, stenosis, and aneurysm of the aorta and branch vessels. In one study, CTA was shown to have a sensitivity of 73% and specificity of 78% to diagnose GCA. CTA has also been used in the diagnosis of TAK with high accuracy. Advantages of CT include its non-invasiveness in comparison to conventional catheter-based angiography and the ability to detect structural lesions with a higher resolution and shorter scanning time than MRA, allowing for more arterial regions to be evaluated in one session. Additionally, CTA is a preferred non-invasive imaging modality to monitor changes in aortic aneurysm morphology over time.”
    “The Role of Vascular Imaging to Advance Clinical Care and Research in Large-Vessel Vasculitis.”  
    Quinn, Kaitlin A, and Peter C Grayson.  
    Current treatment options in rheumatology vol. 5,1 (2019): 20-35
  • “Large vessel vasculitis (LVV) is the most common form of primary vasculitis comprising of giant cell arteritis (GCA), Takayasu’s arteritis (TAK) and idiopathic aortitis. Early diagnosis and treatment of LVV are paramount to reduce the risk of ischemic complications such as visual loss and strokes, vascular stenosis and occlusion, and aortic aneurysm formation. Use of imaging modalities [ultrasound (US), magnetic resonance imaging (MRI), computed tomography (CT) and [18F]-fuorodeoxyglucose positron emission tomography (PET)] has steadily increased to enable assessment of cranial and extracranial arteries, as well as the aorta. These imaging modalities are less invasive, more sensitive and readily available compared to temporal artery biopsy (TAB).”
    EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice summary.  
    Bardi M, Diamantopoulos AP.  
    Radiol Med. 2019 Oct;124(10):965-972. 
  • “Microaneurysms involving medium-sized or small arteries that are often found together with stenotic/occlusive lesions are the hallmark of PAN. Microaneurysms are most frequently identified at renal and splanchnic artery branches (particularly hepatic artery and mesenteric artery). Nonetheless, they are commonly present also in skeletal muscle arteries. Typically, they are saccular or fusiform, and 1–5 mm in diameter.”
    Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa.  
    Hočevar A et al.
    Curr Rheumatol Rep. 2021 Feb 10;23(3):14.
  • “PAN is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries. The diagnosis of this rare disease is primarily based on clinical findings, imaging, and histopathological findings. Different PAN variants have been recognized, and they differ in their etiology as well as in the level of severity and prognosis. Cutaneous PAN usually has a favorable prognosis and requires a less aggressive therapeutic approach, based on nonsteroidal anti-inflammatory drugs to glucocorticoids; the focus of HBV-associated PAN is to control of the viral infection and remove immune complexes. TNF alpha inhibitors are the drugs of choice for PAN associated with ADA2 deficiency. Treatment of idiopathic generalized PAN is guided by disease severity and is in severe cases based on the combination of glucocorticoids and cyclophosphamide. Anti-TNF agents, tocilizumab, or rituximab may be a treatment option in refractory PAN.”
    Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa.  
    Hočevar A et al.
    Curr Rheumatol Rep. 2021 Feb 10;23(3):14.
  • "The stenosis/occlusion and the rupture of inflamed visceral arteries, with subsequent tissue/organ ischemia, damage, dysfunction, and/or bleeding, are the causes of the most profound PAN manifestations. Almost any organ/organ system can be affected by PAN, though pulmonary involvement is uncommon. The most frequently involved organs are the peripheral nervous system and the skin. Cutaneous features include livedo reticularis, subcutaneous nodules, skin infarcts and ulcers, as well as purpura. Mononeuritis multiplex is the most common neurologic manifestation, followed by symmetric polyneuropathy. The involvement of the central nervous system is rare in idiopathic systemic PAN. Gastrointestinal tract inflammation and ischemia often presenting as acute surgical abdomen is one of the most serious PAN manifestations, occurring in up to one-third of cases.”
    Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa.  
    Hočevar A et al.
    Curr Rheumatol Rep. 2021 Feb 10;23(3):14.
  • “Kidneys are commonly affected, with vasculitis of the renal and interlobar arteries, less frequently of the smaller arcuate and interlobular arteries leading to tissue infarctions and microaneurysm rupture. Patients develop micro- or macro-hematuria, and often mild to moderate proteinuria. New onset hypertension is secondary to intrarenal artery involvement and occurs in up to 35% of PAN patients. Renal infarctions or uncontrolled hypertension may lead to chronic renal failure.”
    Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa.  
    Hočevar A et al.
    Curr Rheumatol Rep. 2021 Feb 10;23(3):14.
  • Large Vessel Vasculitis: Takayasu Arteritis
    Takayasu arteritis, which is also known as “aortic arch syndrome” and “pulseless disease”, is an idiopathic inflammatory disease that primarily affects large vessels such as the aorta, major branches of the aorta, and coronary and pulmonary arteries. Pathologically, TA is characterized by panarteritis affecting all three arterial layers, and subsequent scarring of media leading to luminal occlusion
  • Large Vessel Vasculitis: Takayasu Arteritis
    Takayasu arteritis occurs worldwide, but is much more common in Asia. Women are predominantly affected compared to men, with a 7:1 ratio . The American College of Rheumatology states that TA can be suspected when patients have at least three of the following criteria; age < 40 years at disease onset, claudication of the extremities, decreased brachial artery pressure, blood pressure difference of more than 10 mm Hg in between the arms, bruit over the subclavian arteries or aorta, and abnormal arteriographic results.
  • Takayasu Arteritis: Facts
    - Aortic involvement is common in TA; thoracic aorta and its branches are commonly affected, with a 65% prevalence 
    - Pulmonary arteries and coronary arteries could also be affected, with prevalences of 63.3 and 44.4%, respectively
    - In the late phase (occlusive stage), arterial stenosis, occlusion, or aneurysmal dilatation may occur, associated with the mural thickening
  • Large Vessel Vasculitis:  Giant Cell Arteritis
    - Giant cell arteritis (GCA) is another form of large vessel vasculitis affecting the aorta and its large arterial branches. Characteristically, it commonly affects the branches of carotid arteries, vertebral arteries, and temporal arteries, leading to characteristic temporal headaches
    - GCA predominantly affects female patients who are older than 50 years old, and is prevalent in the western countries, in contrast to TA.
  • Medium Vessel Vasculitis: Polyarteritis Nodosa
    - Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium and small arteries without glomerulonephritis, and is unassociated with the anti-neutrophil cytoplasmic antibody (ANCA) 
    - Polyarteritis nodosa affects men twice as often as women in the 5–7th decades. Etiology is unclear, but the hepatitis B virus may play an important role in the development of the disease. Renal involvement is common with an incidence of 70–80%, resulting in proteinuria, hematuria, and hypertension, but not glomerulonephritis.
  • Medium Vessel Disease: Kawasaki Disease
    - Kawasaki disease is a medium vessel vasculitis associated with mucocutaneous lymph node syndrome, and it frequently occurs in infants and children younger than 5 years. Although exact pathogenesis is unknown, it seems likely that infection or autoimmunity play a critical role.
    - Coronary artery involvement is common in patients with KD, therefore myocardial ischemia or infarction frequently occur. CT findings include coronary artery aneurysm, ectasia, premature atherosclerosis and stenosis. A typical braid-like appearance of coronary arteries is one of the characteristics of KD 
  • Small Vessel Vasculitis
    Small vessel vasculitides mainly affect small intraparenchymal arteries, arterioles, capillaries, and venules. They are divided into two categories; ANCA-associated small vessel vasculitis and immune complex-associated small vessel vasculitis.
  • Mimickers of Vasculitis
    - Fibromuscular dysplasia (FMD)
    - Segmental arterial mediolysis
    - Neurofibromatosis type 1
  • Coronary Artery Aneurysms: Causes
    - Atherosclerotic disease
    - Vasculitis (Kawasaki Disease, Takayasu Disease, PAN)
    - Connective Tissue Disease (Marfans, Ehlers-Danlos disease)
    - Infection (Bacterial, mycobacterial, fungal, Lyme disease)
    - IVDA (cocaine, amphetamines)
    - Congenital (FMD)
  • Kawasaki Disease: Facts
    - Occurs in children younger than 5 yrs with peak under age 2 yrs
    - Male > female by 1.5x
    - More common in Asians especially Japanese
    - Acute febrile systemic illness in children
    - AKA mucocutaneous lymph node syndrome
  • Kawasaki Disease: Facts
    - Fever for 5 days or more without a cause and four of the following
    --- Bilateral conjunctival injection
    --- Mucous membrane changes (strawberry tongue)
    --- Extremity abnormalities including erythema of palms or soles, edema of hands or feet
    --- Rash
    --- Cervical lymphadenopathy
  • Kawasaki Disease: Facts
    - Cardiac abnormalities
    --- Pericardial effusion (30%)
    --- Myocarditis
    --- Mitral regurgitation (30%)
    --- Aortitis and aortic regurgitation
    --- Congestive heart failure
    --- Coronary artery aneurysms
  • Kawasaki Disease: Facts
    - Coronary artery aneurysms occur in up to 25% of patients treated with aspirin alone or no treatment
    - Aneurysms develop within 2 weeks (1-4 weeks) with a 2% mortality rate
    - Aneurysms are more common in the proximal than distal coronary artery
    - Aneurysms may thrombosis which results in sudden death
    - Vascular complication rate decreased with proper therapy (aspirin and intravenous immune globulin) to around 5% for aneurysms
  • Vasculitis: Differential Dx
    - PAN
    - SAM (Segmental Arterial Mediolysis)
    - FMD
  • “Segmental arterial mediolysis (SAM) is a non-inflammatory, non-atherosclerotic vasculopathy mostly involving the abdominal arteries. SAM was recently recognized as a more prevalent aetiology of abdominal pain than initially thought by healthcare providers. It is still a commonly missed diagnosis in patients with recurrent emergency room (ER) visits for abdominal pain. Most published case reports in the past have highlighted catastrophic sequelae such as intra-abdominal haemorrhage requiring surgical intervention. We report a case of SAM where the diagnosis was initially missed. After diagnosis, conservative medical management was offered which led to clinical improvement.”
    Segmental Arterial Mediolysis: An Under-Recognized Cause of Chronic Abdominal Pain.
    Chatterjee T, Stephens J, Roy M
    Eur J Case Rep Intern Med. 2020 Jul
  • SAM is a non-inflammatory, non-atherosclerotic vasculopathy of unclear aetiology, with a propensity to involve splanchnic vessels but it can also affect carotid, renal and intracranial vessels. Arterial vasospasm is thought to lead to dissection of arterial walls, although the exact pathogenesis of the disease remains unclear. nother pathophysiological mechanism suggested is vacuolar degeneration leading to disruption of the outer arterial media eventually causing aneurysms and dissections. SAM mainly affects middle-aged and elderly patients. Abdominal pain is the most common clinical presentation, while it can also present as shock due to haemorrhage, haematochezia, chest pain or stroke.”
    Segmental Arterial Mediolysis: An Under-Recognized Cause of Chronic Abdominal Pain.
    Chatterjee T, Stephens J, Roy M
    Eur J Case Rep Intern Med. 2020 Jul
  • “Positive angiographic evidence of disease in the visceral arteries is found in 40-90% of patients with PAN at the time of presentation.Arteries of the extremities and small branches of the aorta are less commonly involved. The typical angiographic feature is the presence of micro-aneurysms. Arterial steno-occlusive lesions are less common.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227
  • “PAN is a focal pan-mural necrotising vasculitis involving small- and medium-sized arteries with multiple organ system involvement to varying degrees, The kidneys are the most common site of involvement (70-80%) with the renal artery most frequently involved, followed by the gastrointestinal tract (GIT), peripheral nerves and skin (50% each) and skeletal muscles (30%). The central nervous system is involved in 10% of cases. Rare sites of involvement are the lung, heart, testicles, and spleen.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227
  • “In conclusion, CT, including CTA, is a comprehensive imaging method that can assist in the diagnosis of PAN in occult cases based on the combination of arterial and parenchymal abnormalities. Knowledge of various CT/CTA abnormalities may obviate the need for angiography and biopsy, which is beneficial considering the invasive nature and potential complications associated with these interventions.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227
  • “None of the CT studies reported arterial changes in PAN besides aneurysms; however, in the present study arterial changes were classified similar to those reported on angi- ography. In their review of the angiographic findings in 56 patients with PAN, Stanson et al.reported occlusive lesions in 39% cases. The spectrum of findings included luminal irregularity, stenosis, and occlusion. Similar changes were found in 11 arteries in the present study; however, in the study of Stanson et al., the SMA was the most common site of involvement followed by the hepatic and splenic arteries. Similarly, ectasia was not reported in CT studies of PAN. Ectasia is less common compared to aneurysms and stenosis and was reported in 12.5% of patients, SMA being the most commonly involved artery.”
    Role of multidetector abdominal CT in the evaluation of abnormalities in polyarteritis nodosa
    M. Singhal et al.
    Clinical Radiology 71 (2016) 222-227
  • “Vasculitic vessel wall changes are usually smoother and more homogeneous than arterio sclerotic lesions and calcifications are less common. In addition, CTA-derived multiplanar reformations enable accurate diameter measurements in case of late complications of vasculitis such as aneurysms or stenoses.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • "Combining PET with CT (PET/CT) or CTA gives additional information on wall thickness and luminal changes. When PET/CT is used for imag- ing of vasculitis, [18F]-fluorodeoxyglucose (FDG) a pronounced enhancement of vessel walls is intravenously applied FDG-PET/CT detects glucose uptake from high glycolytic activity of inflammatory cells in inflamed arteries, resulting in a pronounced enhancement of vessel wall.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • “The 2 major vasculitis entities affecting the large vessels are GCA and Takayasu’s arteritis (TAK). Even though GCA and TAK have some distinctive imaging features, they are both systemic inflammatory diseases, which might result in variable involvement patterns.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • “Imaging features of TAK and GCA overlap and are not indicative of 1 disease. Stenosis, occlu- sion, ectasia, mural and surrounding edema, mural thickening, and contrast enhancement in MR imaging or CT, and FDG uptake in PET/CT can occur in either disease. The main criteria for diagnosis of either TAK or GCA consist of imaging-based identification of different patterns of vessel involvement, in combination with demographic and clinical information.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • "Segmental involvement patterns of the supra aortic vessels, especially of the cranial arteries (superficial temporal and occipital artery), are classic features of GCA. Notably, in up to 40% of patients with extracranial GCA there is no involvement. Also, GCA tends to have a predilection for the axillary arteries.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • "Takayasu’s arteritis (TAK) is less common than GCA and affects younger patients ( 40 years) with a female predominance. It was originally diagnosed in Japan and is more common in patients of Asian and African ancestry than European ancestry.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • “A broad range in clinical signs and symptoms of at least 1 month’s duration are considered charac- teristic of TAK: limb claudication, pulselessness or pulse differences in limbs, an unobtainable or sig- nificant blood pressure difference (>10 mm Hg systolic blood pressure in the limb), fever, neck pain, transient amaurosis, blurred vision, syncope, dyspnea or palpitations. TAK and GCA overlap in terms of involvement of the aorta and its major branches. TAK may extend from the aortic arch and its major branches from the carotids to the external iliac arteries, including the pulmonary arteries.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • "Late complications (TK) result in an occlusive stage with arterial stenosis, occlusion, or aneurysmal dilatation, which may lead to extensive collateral vessels.Therefore, MRA and CTA should include the aorta and its major branches from the carotid bifurcation to the iliac arteries in coronal acquisition, as well as the axillary and brachial arteries.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • "PAN is a necrotizing arteritis of medium or small arteries, which is typically antineutrophil cytoplasmic antibody negative. PAN is generally found in all age groups, but most commonly in those between 50 and 70 years of age, occurring twice as often in men compared with women.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • “Characteristic imaging findings of PAN include multiple microaneurysms (1–5 mm) often involving the renal, mesenteric, and hepatic artery branches. Because different stages of the inflammatory process occur simultaneously, vessels with acute necrotizing lesions, alongside fibrotic or healing vessels, might be present. Therefore microaneurysms often coexist with stenotic lesions.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • "KD is associated with mucocutaneous lymph node syndrome and predominantly affects medium and small arteries in infants and children less than 5 years of age. It is more prevalent in Asian populations and has a male dominance.”
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • “The coronary arteries are often involved in KD and coronary artery aneurysms develop as a result of coronary vasculitis in about 15% to 25% of untreated patients. Coronary artery aneurysms can be classified according to their size (small, <5 mm; medium, 5–8 mm; and large, >8 mm) and shape (saccular or fusiform). Large coronary artery aneurysms are associated with a higher risk of complications such as rupture, thrombosis, and stenosis, which possibly lead to myocardial infarction and death."
    Radiologic Imaging in Large and Medium Vessel Vasculitis
    Weinrich JM et al.
    Radiol Clin N Am 58 (2020) 765–779
  • “Behcet's disease (BD) is a chronic inflammatory disorder, with vasculitis underlying the pathophysiology of its multisystemic effects. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognized that cardiac involvement and arterial complications are also important aspects of the course of the disease. Cardiac lesions include pericarditis, endocarditis, intracardiac thrombosis, myocardial infarction, endomyocardial fibrosis, and myocardial aneurysm.”
    Cardiac manifestations in Behcet's disease
    Selami Demirelli et al.
    Intractable Rare Dis Res. 2015 May; 4(2): 70–75.
  • "The morphological basis of the systemic manifestations in BD, including cardiovascular involvement, is vasculitis . More specifically, some pathologists consider perivascular structures as the main target of T lymphocyte-mediated immune reactions, and perivasculitis as an essential part of the vasculopathy in BD. The venous and arterial wall lesions attract cytokinergic and neutrophilic reactions. Activated neutrophils cause destructive effects by excessive production of superoxide anion radicals and lysosomal enzymes. Neutrophilic infiltration and advanced vascular wall destruction with aneurysm formation cause local blood flow abnormalities.”
    Cardiac manifestations in Behcet's disease
    Selami Demirelli et al.
    Intractable Rare Dis Res. 2015 May; 4(2): 70–75.
  • "Coronary aneurysms may be seen during angiography procedures in patients with BD. Some of these aneurysms are asymptomatic, whereas others manifest with acute coronary syndrome. These aneurysms are isolable, and most are evident together with coronary stenosis and are sometimes seen together with several arterial aneurysms.”
    Cardiac manifestations in Behcet's disease
    Selami Demirelli et al.
    Intractable Rare Dis Res. 2015 May; 4(2): 70–75.
  • “Behcet's disease (BD) is a multisystem vasculitis of unknown etiology, characterized by recurrent urogenital ulceration, cutaneous eruptions, uveitis, arthritis and vasculitis. Less commonly, coronary arteries are involved, with potential serious consequences.”
    PREVALENCE AND PREDICTORS OF CORONARY ARTERY DISEASE IN BEHCET'S DISEASE
    Jalaj Garg et al. 
    Journal of the American College of Cardiology
    Volume 63, Issue 12 Supplement, April 2014
  • Angiographic Findings in Patients with PAN



    Polyarteritis Nodosa: Spectrum of Angiographic Findings
    Anthony W. Stanson et al.
    RadioGraphics 2001; 21:151-159
  • PAN Vascular Findings
    - occlusive lesions (luminal irregularities resulting in reduction of caliber, stenosis, or occlusion)
    - microaneurysms
    - Polyarteritis Nodosa: Spectrum of Angiographic Findings
    - Anthony W. Stanson et al.
    - RadioGraphics 2001; 21:151-159
  • Differential Dx for Multiple Renal Artery Aneurysms on CT
    - polyarteritis nodosa
    - rheumatoid vasculitis,
    - systemic lupus erythematosus,
    - Churg- Strauss syndrome
    - IVDA
  • “As seen in this review of the spectrum of angiographic findings in PAN, a variety of arterial beds may be affected and a variety of lesions may be seen. Skeletal muscle arterial disease was seen in nearly one-third of our patients. The lesions seen most frequently were occlusive. The presence of aneurysms increases the specificity of the diagnosis of PAN, but in their absence other arterial lesions such as luminal irregularities, stenoses, and occlusions can suggest the diagnosis. In a patient without fulminant disease but in whom clinical suspicion is firm, comprehensive angiography may lead to the diagnosis of PAN on the basis of the constellation of angiographic findings.”
    Polyarteritis Nodosa: Spectrum of Angiographic Findings
    Anthony W. Stanson et al.
    RadioGraphics 2001; 21:151-159
  • “Many of the clinical symptoms are related to organ ischemia secondary to arterial branch occlusions. Aneurysm rupture is a less common cause of pain. Arthralgias are noted in 50% of patients as are peripheral neuropathies (mononeuritis multiplex), which are often symptomatic early. Renal involvement including proteinuria and hypertension are found in 75%. Branch vessel occlusions can lead to multiple renal infarcts. Abdominal pain from ischemia or infarction secondary to occlusive lesions is the most common gastrointestinal complaint. Abdominal pain may also be caused by rupture of an aneurysm in one of the viscera: liver, kidney, or mesentery. Cutaneous lesions include palpable purpura, infections, and ischemic ulcers. Muscle pain and limb claudication may occur.”
    Polyarteritis Nodosa: Spectrum of Angiographic Findings
    Anthony W. Stanson et al.
    RadioGraphics 2001; 21:151-159
  • “Polyarteritis nodosa (PN) is a systemic necrotizing vasculitis of medium and small arteries, and is typically found in middle-aged men. The most common clinical symptoms are persistent fever, weight loss, and polyarthragia. As these symptoms are nonspecific, diagnosing PN is sometimes difficult and delayed. The kidney is the most commonly involved, and multiple small wedge-shaped less-enhanced areas are typically seen on enhanced CT. These are considered to be multiple bilateral renal cortical infarctions due to vasculitis of the interlobar arteries and arcuate arteries.”
    Renal involvement of polyarteritis nodosa: CT and MR findings
    Ozaki, K., Miyayama, S., Ushiogi, Y. et al.
    Abdom Imaging (2009) 34: 265. https://doi.org/10.1007/s00261-008-9377-7
  • Some studies reported that multiple small wedge-shaped less-enhanced areas were observed on enhanced CT, which were multiple bilateral renal cortical infarctions from vasculitis of the interlobar arteries and the arcuate arteries. In addition, perirenal hematoma by aneurysmal rupture, and small microaneurysms are sometimes observed.
    Renal involvement of polyarteritis nodosa: CT and MR findings
    Ozaki, K., Miyayama, S., Ushiogi, Y. et al.
    Abdom Imaging (2009) 34: 265. https://doi.org/10.1007/s00261-008-9377-7
  • “Most aneurysms are 2–10 mm in diameter. In the present study, multiple microaneurysms, 2–3 mm in diameter, were observed in all patients. However, it is important to note that renal microaneurysms are not specific to PN. They may be demonstrated in other systemic necrotizing vasculitis, drug abuse, and bacterial endocarditis. The specificity of angiographic findings of microaneurysm becomes much higher when the clinical symptoms are supportive.”
    Renal involvement of polyarteritis nodosa: CT and MR findings
    Ozaki, K., Miyayama, S., Ushiogi, Y. et al.
    Abdom Imaging (2009) 34: 265. https://doi.org/10.1007/s00261-008-9377-7

  • Hur JH, Chun EJ, Kwag HJ, Yoo JY, Kim HY, Kim JJ, Lee KW. CT Features of Vasculitides Based on the 2012 International Chapel Hill Consensus Conference Revised Classification. Korean J Radiol. 2017 Sep-Oct;18(5):786-798.

  • Hur JH, Chun EJ, Kwag HJ, Yoo JY, Kim HY, Kim JJ, Lee KW. CT Features of Vasculitides Based on the 2012 International Chapel Hill Consensus Conference Revised Classification. Korean J Radiol. 2017 Sep-Oct;18(5):786-798.

  • Hur JH, Chun EJ, Kwag HJ, Yoo JY, Kim HY, Kim JJ, Lee KW. CT Features of Vasculitides Based on the 2012 International Chapel Hill Consensus Conference Revised Classification. Korean J Radiol. 2017 Sep-Oct;18(5):786-798.
  • Takayasu Arteritis: Facts
    - Takayasu arteritis, which is also known as “aortic arch syndrome” and “pulseless disease”, is an idiopathic inflammatory disease that primarily affects large vessels such as the aorta, major branches of the aorta, and coronary and pulmonary arteries. Pathologically, TA is characterized by panarteritis affecting all three arterial layers, and subsequent scarring of media leading to luminal occlusion.
    - Takayasu arteritis occurs worldwide, but is much more common in Asia . Women are predominantly affected compared to men, with a 7:1 ratio. The American College of Rheumatology states that TA can be suspected when patients have at least three of the following criteria; age < 40 years at disease onset, claudication of the extremities, decreased brachial artery pressure, blood pressure difference of more than 10 mm Hg in between the arms, bruit over the subclavian arteries or aorta, and abnormal arteriographic results.
  • Takayasu Arteritis: Facts
    CT findings of TA differ depending on the stage. In the early phase (active stage), concentric mural thickening of the involved arteries is characteristic. On pre-contrast CT scanning, the mural thickening is of high attenuation compared with the lumen, while on the post-enhanced CT angiographic images, it exhibits a “double ring sign”. Double ring sign is typically shown in the venous phase, as an inner low attenuating wall surrounded by an outer high attenuating wall. In the late phase (occlusive stage), arterial stenosis, occlusion, or aneurysmal dilatation may occur, associated with the mural thickening . Extensive vascular calcification and collateral vessels may be observed with luminal narrowing.
  • Giant Cell Arteritis: Facts
    Giant cell arteritis (GCA) is another form of large vessel vasculitis affecting the aorta and its large arterial branches. Characteristically, it commonly affects the branches of carotid arteries, vertebral arteries, and temporal arteries, leading to characteristic temporal headaches. A microscopic exam reveals vasculitis with mononuclear infiltrates or granulomas in the active stage and vessel wall fibrosis in the chronic stage.
  • Giant Cell Arteritis: Facts
    Clinical differentiation between GCA and TA is sometimes difficult, but GCA predominantly affects female patients who are older than 50 years old, and is prevalent in the western countries, in contrast to TA. Pathologically, TA is commonly associated with extensive intimal and adventitial fibrosis with resultant luminal narrowing, whereas GCA is more commonly associated with extensive medial inflammation, necrosis, and formation of aortic aneurysm. “Skip lesion”, or aortic inflammation, is also more common in GCA.
  • Polyarteritis Nodosa: Facts
    - Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium and small arteries without glomerulonephritis, and is unassociated with the anti-neutrophil cytoplasmic antibody (ANCA). Absence of ANCA is a distinguishing point from microscopic polyangiitis (MPA), which is an ANCA-associated small vessel vasculitis that shares similar pathologic and radiologic findings with PAN.
    - Polyarteritis nodosa affects men twice as often as women in the 5–7th decades. Etiology is unclear, but the hepatitis B virus may play an important role in the development of the disease. Renal involvement is common with an incidence of 70–80%, resulting in proteinuria, hematuria, and hypertension, but not glomerulonephritis. Other involvements are the gastrointestinal tract and skin at an incidence rate of 50%; skeletal muscles and mesentery at an incidence rate of 30%; and the central nervous system at an incidence rate of 10%.
    - Characteristic imaging findings of PAN are microaneurysms of medium or small arteries, often involving the renal arteries.
  • Polyarteritis Nodosa: Facts
    - Small vessel vasculitides mainly affect small intraparenchymal arteries, arterioles, capillaries, and venules. They are divided into two categories; ANCA-associated small vessel vasculitis and immune complex-associated small vessel vasculitis.
    - Anti-neutrophil cytoplasmic antibody is an antibody against intracellular antigens in neutrophils and monocytes. According to immunofluorescent staining pattern, there are cytoplasmic ANCA (c-ANCA) and perinuclear ANCA (p-ANCA). Granulomatosis with polyangiitis (GPA) is a c-ANCA (+) vasculitis, whereas eosinophilic GPA (EGPA) or MPA are characteristic of p-ANCA (+) vasculitis. Although ANCA is an important characteristic of ANCA-associated vasculitis, its positive predictive value is only around 45%. This means that ANCA negativity does not exclude the possibility of ANCA-associated vasculitis.
  • Behçet’s Disease: Facts
    - Behçet's disease is a chronic, relapsing autoinflammatory condition involving multiple organs. Clinically, Behçet's disease manifests as recurrent oral or genital aphthous ulcers, and inflammatory lesions of the cutaneous, ocular, articular, gastrointestinal, and central nervous system. It is most prevalent in the 3rd and 4th decades of life, and is more common in males.
    - Vascular involvement occurs in 5–30% of Behçet's disease cases, and it can be divided into three types: venous thrombosis, arterial aneurysm and aortic occlusion. Venous thrombosis is associated with a prevalence range of 10–30%. Deep vein thrombosis of the lower extremities is the most common finding, followed by superficial thrombophlebitis after venipuncture. Aneurysms, which are frequently seen in the aorta, pulmonary, subclavian, and popliteal arteries, can be the most serious complication of Behçet's disease. Saccular types are more frequent than fusiform aneurysms. In case of pulmonary involvement, pulmonary artery aneurysm is the most common finding with a prevalence of 1–10%, and tends to be multiple and bilateral.
  • Differential Diagnosis of Vasculitis: Facts
    The presence of known etiology or associated systemic disease can be helpful for the differential diagnosis of vasculitis. Systemic lupus erythematosus or rheumatoid arthritis are systemic inflammatory disorders, which may lead to inflammation of the cardiovascular system. IgG4-related sclerosing disease is characterized by idiopathic inflammatory lesions infiltrated with numerous IgG4-positive plasma cells. IgG4-related periarteritis predominantly involves the abdominal aorta, iliac vessels and thoracic aorta. CT findings include arterial wall thickening, homogeneous enhancement at the delayed phase of contrast enhanced CT, luminal change (mostly dilation and rarely stenosis), and exaggerated atherosclerotic change. An elevated serum IgG4 level and associated organ damage are essential in discerning this disease from other vasculitides. A few cases of IgG4-related periarteritis affecting the coronary arteries have been reported, which appeared as soft tissue encasing the coronary artery with or without luminal narrowing, wall calcification, and aneurysm.
  • Differential Diagnosis of Vasculitis: Facts
    Multidetector CT is a useful noninvasive imaging modality for the evaluation of vasculitis and vasculitis mimics because CT can provide the information of the vessel wall change and other accompanied findings. Although CT features of various vasculitis are often overlapping, CT features via consideration of the involved vessel type, location, morphology, and associated systemic disease, can be useful in narrowing down the differential diagnosis .Getting familiar with CT features will help radiologists to establish appropriate diagnosis for vasculitis.
  • "Kawasaki’s disease (KD) is a vasculitis that predominantly affects children and can lead to the development of coronary artery aneurysms. These aneurysms can subsequently thrombose and occlude, which may lead to chest pain and other signs and symptoms of acute coronary syndrome in young patients. Coronary CT angiography, including 3D visualization techniques, is a common modality used in the follow-up of KD patients."
    Evaluation of Kawasaki’s disease-associated coronary artery aneurysms with 3D CT cinematic rendering
    Rowe SP, Zimmerman SL, Johnson PT, Fishman EK
    Emergency Radiology (2018) 25:449–453
  • "The most important potential complication of KD is inflammatory damage to the coronary arteries, which classically leads to the formation of coronary artery aneurysms. These aneurysms will often undergo vascular remodeling and partial
    thrombosis in order to produce a psuedonormal vascular lumen. However, progressive thrombosis and other stenotic processes such as fibrosis can, over time, precipitate cardiac ischemia that can lead to emergency room presentations for chest pain or other signs of acute coronary syndrome and necessitate invasive and/or surgical interventions."
    Evaluation of Kawasaki’s disease-associated coronary artery aneurysms with 3D CT cinematic rendering
    Rowe SP, Zimmerman SL, Johnson PT, Fishman EK
    Emergency Radiology (2018) 25:449–453
  • CR differs from traditional VR in making use of a global lighting model that more realistically takes into account the interactions of propagating photons with the component materials of the imaged volume. As a result, this method enhances surface detail and creates lifelike shadowing effects in order to generate truly photorealistic visualizations from standard CT acquisition volumetric data."
    Evaluation of Kawasaki’s disease-associated coronary artery aneurysms with 3D CT cinematic rendering
    Rowe SP, Zimmerman SL, Johnson PT, Fishman EK
    Emergency Radiology (2018) 25:449–453
  • " While the advantages of this new technique are still under investigation, potential applications include improved visualization of complex anatomic structures that obviates the need for expensive 3D printing, better display of soft tissue texture that may improve detection and characterization of subtle lesions, improved pre-operative planning, and better trainee and patient engagement."
    Evaluation of Kawasaki’s disease-associated coronary artery aneurysms with 3D CT cinematic rendering
    Rowe SP, Zimmerman SL, Johnson PT, Fishman EK
    Emergency Radiology (2018) 25:449–453
  • In this case series, we have demonstrated the ability of CR visualizations to effectively convey key anatomic information related to coronary artery aneurysms in patients with history of KD. While the role of imaging in following patients with KD has been previously investigated, this is the first reported experience with the novel CR visualization methodology, and these examples demonstrate the potential of this technique for evaluating such complex vascular pathologic states.
    Evaluation of Kawasaki’s disease-associated coronary artery aneurysms with 3D CT cinematic rendering
    Rowe SP, Zimmerman SL, Johnson PT, Fishman EK
    Emergency Radiology (2018) 25:449–453
  • "As with other 3D visualization methods, CR would be expected to provide a global overview of the disease process that is not easily appreciated on 2D axial or multi-planar reformatted images. In particular, this may help to identify relatively distal, small caliber sites of aneurysmal enlargement, which stand out with high contrast on the 3D images but can be subtle when only 2D images are viewed."
    Evaluation of Kawasaki’s disease-associated coronary artery aneurysms with 3D CT cinematic rendering
    Rowe SP, Zimmerman SL, Johnson PT, Fishman EK
    Emergency Radiology (2018) 25:449–453
  • “Vasculitis represents a range of different diseases that result in inflammation and necrosis of the blood vessels, with different types of vasculitis showing involvement of different types of vasculature. In the past, vasculitis has been divided into 3 categories depending on the caliber of vessels involved: large vessel vasculitis, which involves the aorta and its major branches (eg, Takayasu arteritis, giant cell arteritis); medium vessel arteritis (eg, polyarteritis nodosa [PAN], Kawasaki disease); and small vessel vasculitis (eg, lupus vasculitis, Henoch-Schönlein purpura [HSP], Wegner granulomatosis, Behcet disease).”


    Computed Tomography Angiography of the Small Bowel and Mesentery 
Raman SP, Fishman EK
Radiol Clin N Am 54 (2016) 87–100
  • “Although most visceral artery dissections merely reflect the extension of an aortic dissection into a branch vessel, isolated visceral artery dissections are still thought to be rare (despite their increasing diagnosis), and almost always reflect an underlying intrinsic weakness of the vessel wall caused by such entities as fibromuscular dysplasia (FMD), cystic medial necrosis, collagen vascular disease, vasculitis, Marfan, or Ehlers-Danlos. The most commonly involved vessel is the SMA, although any visceral artery can theoretically be involved. In most cases, isolated visceral artery dissections occur close to the vessel ostium, possibly as a result of shear stresses to the vessels in this location.”


    Computed Tomography Angiography of the Small Bowel and Mesentery 
Raman SP, Fishman EK
Radiol Clin N Am 54 (2016) 87–100
  • “Classically diagnosed in elderly patients who have abdominal pain after meals with signs of anorexia and chronic weight loss, chronic mesenteric ischemia is much less common than acute mesenteric ischemia.Most cases are attributable to atherosclerotic narrowing of the origin of the major mesenteric arteries, and symptoms typically result only in patients who have a significant stenosis in at least 2 of the 3 major mesenteric arteries (celiac, SMA, and IMA). However, given that atherosclerotic disease is widespread in the elderly population, the diagnosis of this entity requires not only correlation with appropriate clinical symptoms but also visualization of collateral pathways (eg, celiac-SMA collaterals via the pancreaticoduodenal arcade and SMA-IMA collaterals via the arc of Riolan and marginal artery of Drummond). Although these collateral pathways can compensate for significant stenoses over long periods of time, symptoms usually develop when blood flow via these collaterals is no longer sufficient to supply the bowel.”


    Computed Tomography Angiography of the Small Bowel and Mesentery 
Raman SP, Fishman EK
Radiol Clin N Am 54 (2016) 87–100
  • “Classically diagnosed in elderly patients who have abdominal pain after meals with signs of anorexia and chronic weight loss, chronic mesenteric ischemia is much less common than acute mesenteric ischemia.Most cases are attributable to atherosclerotic narrowing of the origin of the major mesenteric arteries, and symptoms typically result only in patients who have a significant stenosis in at least 2 of the 3 major mesenteric arteries (celiac, SMA, and IMA).”


    Computed Tomography Angiography of the Small Bowel and Mesentery 
Raman SP, Fishman EK
Radiol Clin N Am 54 (2016) 87–100
  • Vasculitis of the Abdominal Aorta: Facts
    • Giant cell arteritis (GCA) and Takayasu arteritis (TA) are the most common large vessel vasculitides to have imaging findings on CTA. Both are systemic large vessel granulomatous inflammatory processes.
    • Both diseases are idiopathic inflammatory disorders with various postulated environmental, autoimmune, and genetic causes.
    • GCA typically affects older individuals of northern European heritage, whereas TA is classically associated with younger females of Asian heritage.
    • Clinical manifestations of disease include abnormal or absent pulses as well as symptoms related to occlusion or narrowing of large vessels (eg, mesenteric ischemic symptoms if the splanchnic vasculature is involved or renal failure if the renal arteries are involved).

    Computed Tomographic Angiography of the Abdominal Aorta Hansen NJ Radiol Clin N Am 54 (2016) 35–54
  • Vasculitis of the Abdominal Aorta: Facts
    • Giant cell arteritis (GCA) and Takayasu arteritis (TA) are the most common large vessel vasculitides to have imaging findings on CTA. Both are systemic large vessel granulomatous inflammatory processes.
    • Both diseases are idiopathic inflammatory disorders with various postulated environmental, autoimmune, and genetic causes.

    Computed Tomographic Angiography of the Abdominal Aorta Hansen NJ Radiol Clin N Am 54 (2016) 35–54
  • Vasculitis of the Abdominal Aorta: Facts
    • GCA typically affects older individuals of northern European heritage, whereas TA is classically associated with younger females of Asian heritage.
    • Clinical manifestations of disease include abnormal or absent pulses as well as symptoms related to occlusion or narrowing of large vessels (eg, mesenteric ischemic symptoms if the splanchnic vasculature is involved or renal failure if the renal arteries are involved).

    Computed Tomographic Angiography of the Abdominal Aorta Hansen NJ Radiol Clin N Am 54 (2016) 35–54
  • “Lower extremity CTA is performed for a number of clinical indications, including peripheral artery disease, trauma, assessment of variant anatomy and congenital malformations, vasculitis, and surgical planning.”


    Computed Tomography Angiography of the Lower Extremities 
Cook TS
Radiol Clin N Am 54 (2016) 115–130
  • “Acute thromboembolic disease may occur as a result of embolism originating in the heart or in a more proximal diseased segment of vessel, or thrombosis of a vessel, bypass graft, or lower extremity aneurysm. Patients present with symptoms of acute limb ischemia that can progress over hours to days, and include pain, paresthesias, pallor, pulselessness, and paralysis.The goal of treatment is reperfusion of the affected limb, which is usually achieved with a combination of anticoagulation and either surgery or endovascular intervention; in rare cases, anticoagulation alone is used.”


    Computed Tomography Angiography of the Lower Extremities 
Cook TS
Radiol Clin N Am 54 (2016) 115–130
  • CT Angiography of the Upper Extremities: Applications
    - Atherocsclerotic disease
    - Thromboembolic disease
    - Aortic dissection
    - Vasculitis
    - Small artery vasculitis
    - Fibromuscular dysplasia
    - Extrinsic compression syndrome
    - Connective tissue disorders
    - Preprocedural planning and postprocedural evaluation
  • “ Takayasu arteritis, giant cell arteritis and thromboangitis obliterans (Buerger disease) are the most common types of vasculitides that affect upper extremity vessels.”
    CT Angiography of the Upper Extremity Arterial System: Part 2-Clinical Applications Beyond Trauma Patients
    Bozlar U et al.
    AJR 2013; 201:753-763
  • “Segmental arterial mediolysis (SAM) is a rare vasculopathy of unknown etiology characterized by disruptioSegmental arterial mediolysis (SAM) is a rare vasculopathy of unknown etiology characterized by disruption of the arterial medial layer, with resultant susceptibility to vessel dissection, hemorrhage, and ischemia. n of the arterial medial layer, with resultant susceptibility to vessel dissection, hemorrhage, and ischemia.”
    Clinical diagnosis of segmental arterial mediolysis: Differentiation from vasculitis and other mimics
    Baker-LePain JC et al
    Arthritis Care Res 2010, 62: 1655–1660. 
  • “ Although the abdominal visceral arteries are most frequently affected in SAM , any vessel may be involved, including the retroperitoneal , intracranial , and coronary arteries. The histopathologic changes begin with vacuolar degeneration of smooth muscle cells in the arterial media, followed by fibrin deposition at the medial–adventitial junction . This in turn predisposes to dissecting aneurysms.”
    Clinical diagnosis of segmental arterial mediolysis: Differentiation from vasculitis and other mimics
    Baker-LePain JC et al
    Arthritis Care Res 2010, 62: 1655–1660. 
  • “ The angiographic appearance of SAM is variable, ranging from arterial dilation to aneurysm formation (single or multiple) to stenoses or occlusion, frequently with dissection . Correspondingly, symptoms arise both from stenoses and occlusions (e.g., postprandial pain from intestinal ischemia) and from dissections and aneurysms (e.g., sudden and catastrophic intraperitoneal bleeding).”
    Clinical diagnosis of segmental arterial mediolysis: Differentiation from vasculitis and other mimics
    Baker-LePain JC et al
    Arthritis Care Res 2010, 62: 1655–1660. 
  • “The differential diagnosis of SAM includes atherosclerosis, fibromuscular dysplasia (FMD), infection (e.g., mycotic aneurysm and endocarditis), connective tissue diseases (e.g., Behçet's disease and polyarteritis nodosa [PAN]), neurofibromatosis, and inherited defects in vessel wall structural proteins (e.g., type IV Ehlers-Danlos syndrome and Marfan's syndrome). ”
    Clinical diagnosis of segmental arterial mediolysis: Differentiation from vasculitis and other mimics
    Baker-LePain JC et al
    Arthritis Care Res 2010, 62: 1655–1660. 
  • “In summary, SAM is a rare but important cause of unexplained vascular lesions in patients in whom other inflammatory, infectious, or heritable diseases have been ruled out. The diagnosis should be considered when a patient presents with unexplained acute-onset abdominal pain with or without intraabdominal bleeding. SAM should also be kept in mind when aneurysms, stenoses, and occlusions are identified in medium-sized and large vessels, especially when these lesions are limited to a single anatomic location.”
    Clinical diagnosis of segmental arterial mediolysis: Differentiation from vasculitis and other mimics
    Baker-LePain JC et al
    Arthritis Care Res 2010, 62: 1655–1660. 
  • “ The discrimination of SAM from systemic inflammatory vasculitides is particularly important, since corticosteroids and immunosuppressive agents, which are crucial in the treatment of the inflammatory vasculitides, have no proven benefit in SAM. Without any evidence of an inflammatory etiology, the use of immunosuppressive regimens in SAM exposes the patient to undue risks, including infection and poor wound healing, and could possibly worsen the prognosis. Treatment of SAM involves embolization, surgical bypass, or resection of the injured arteries.”
    Clinical diagnosis of segmental arterial mediolysis: Differentiation from vasculitis and other mimics
    Baker-LePain JC et al
    Arthritis Care Res 2010, 62: 1655–1660.
  • Differential Diagnosis
    - SLE
    - Churg Strauss Syndrome
    - Vasculitis from drug abuse
    - Microscopic polyangitis
  • CTA Findings in PAN
    - Multiple aneurysms of varying sizes
    - Smooth narrowing of vessels; stenosis and occlusions of larger vessels
    - Thickening of wall of medium sized vessels
    - Aneurysms may be associated with hemorrhage
    - Bowel wall thickening often with associated strictures or perforation
  • Polyarteritis Nodosa: Facts
    - Most common in 5th-7th decade of life
    - More common in males (2-1)
    - Survival at 5 yrs less than 15% in fulminant disease
    - 50% of patients with abdominal involvement develop acute surgical abdomen with mortality of 12.5%
  • Polyarteritis Nodosa: Clinical Presentation
    Subacute presentation with weight loss, FUO, malaise
    Acute presentation could include;

    - Renal insufficiency or hemorrhage
    - Abdominal pain including ischemia, infarction, or perforation
    - Palpable purpura or ulcerations
  • Polyarteritis Nodosa (PAN): Facts
    - Systemic vasculitis causing necrotizing inflammation of small and medium sized vessels, resulting in microaneurysms, occlusions and strictures
    - Kidney is most commonly involved (70-80% of cases)
    - GI tract involved in up to 50% of cases
    - Muscle, skin and CNS are often involved
  • Secondary Vasculitides
    - Sarcoidosis
    - Retroperitoneal fibrosis
    - Inflammatory aneurysm
  • Thromboangitis obliterans: Facts
    - Usually involves young males
    - Strong association with smoking
    - Usually involves the extremities
    - Can lead to superficial thrombophlebitis, raynauds phenomenon, and gangrene
  • Behcets disease: facts
    - Chronic inflammatory vascular disease characterized by recurrent oral and genital aphthous ulcers
    - Arteries of all sizes can be involved and lead to dissection, aneurysm or thrombosis
    - Large vessels involved in up to 25% of cases
  • Small Vessel Vasculitis
    - Wegeners granulomatosis
    - Churg-Strauss syndrome
    - Behcets disease
    - Thromboangitis obliterans
    - Cerebral vasculitis
  • Small Vessel Vasculitis
    - Wegeners granulomatosis
    - Churg-Strauss syndrome
    - both are ANCA associated small vessel vasculities (ANCA-antineutrophilic cytoplasmic antibody)
  • Kawasaki’s Disease: Facts
    - Patients usually younger than age 5 years
    - Involves medium sized vessels
    - Key area of involvement is the coronary arteries where up to 25% of patients may develop aneurysms
  • Medium Sized Vessels
    - Kawasaki’s disease
  • Takayasu’s Arteritis: Facts
    - Usually involves aorta and its main branches
    - Usually in woman
    - More common in Asia
    - May also involve pulmonary arteries and coronary arteries
    - Stenosis often occurs proximately and skip lesions do occur
  • Giant Cell Arteritis: Facts
    - Immune mediated disease characterized by granulomatous infiltration of medium and large arteries
    - Most common presentation Is headache
    - More common in woman usually in the 50’s
    - Vessel wall thickening of 1.5 mm or greater and usually symmetric
  • Large Vessel Vasculitides
    - Giant Cell Arteritis
    - Takayasu’s Arteritis
  • "Large and medium sized vessels are affected by many primary and secondary inflammatory disorders or mimicking conditions, often preventing similar imaging characteristics. Knowledge of distinctive imaging features such as location, length, and degree of vessel wall enhancement as well as the particular clinical settings will help the clinician in appropriately narrowing down the differential diagnosis." Imaging of Primary and Secondary Inflammatory Disease Involving Large and Medium Sized Vessels and Their Potential mimics: A Multitechnique Approach
    Spira D et al.
    AJR 2010;194:848-858

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