Imaging Pearls ❯ Vascular ❯ IgG4 Related Disease
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- “IgG4-related cardiovascular disease (CVD) such as aortitis, arteritis, periaortitis, periarteritis, and inflammatory aneurysm, have increased in the last 10 years. The association of inflammatory aortic aneurysm and IgG4-related disease has been sporadically documented .The clinical presentation of this heterogeneous group of disorders varies on the basis of the involvement of the aorta or coronary arteries. Coronary involvement can cause coronary stenosis or aneurysm, which might cause myocardial infarction or sudden cardiac death.”
IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries
Noriko Oyama-Manabe et al.
RadioGraphics 2020; 40:E29–E32 - “The features of IgG4-related CVD on contrast material–enhanced CT images include diffuse or partial arterial wall thickening (>2 mm) and homogeneous wall enhancement. We reported that 41% of patients who definitely had IgG4-related disease exhibited signs and symptoms of IgG4-related aortitis and/or periaortitis, and 80% of these patients had multiple–vascular region involvement.”
IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries
Noriko Oyama-Manabe et al.
RadioGraphics 2020; 40:E29–E32 - "The iliac arteries (35% of cases) and infrarenal abdominal aorta (33% of cases) were the most commonly affected in patients with IgG4-related CVD, followed by the thoracic aorta and first branches of the thoracic aorta (8% of cases), suprarenal abdominal aorta (6% of cases), and first branches of the abdominal aorta (5% of cases). Moreover, the specific distribution of IgG4-related CVD could be differentiated from other large vessel vasculitis, such as Takayasu disease and giant cell arteritis, which mainly affect the thoracic aorta. IgG4-related CVD can occasionally manifest as masses with irregular margins that may or may not be associated with luminal dilatation or stenosis.”
IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries
Noriko Oyama-Manabe et al.
RadioGraphics 2020; 40:E29–E32 - “A research group from Japan proposed the comprehensive criteria for diagnosing IgG4- related disease in routine clinical practice: (a) characteristic diffuse or localized swelling or the presence of masses in one or more organs at clinical examination; (b) elevated serum IgG4 concentration levels (≥135 mg/dL [≥1.35 g/L])at hematologic examination; and (c) marked lymphoplasmacytic infiltration, storiform fibrosis, and infiltration of IgG4-positive plasma cells into organs at histopathologic examination. The diagnosis of IgG4-related disease is definitive when all three criteria are met, probable when the first and third criteria are met, and possible when the first and second criteria are met.” IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries Noriko Oyama-Manabe et al. RadioGraphics 2020; 40:E29–E32
- “A research group from Japan proposed the comprehensive criteria for diagnosing IgG4- related disease in routine clinical practice: (a) characteristic diffuse or localized swelling or the presence of masses in one or more organs at clinical examination; (b) elevated serum IgG4 concentration levels (≥135 mg/dL [≥1.35 g/L])at hematologic examination; and (c) marked lymphoplasmacytic infiltration, storiform fibrosis, and infiltration of IgG4-positive plasma cells into organs at histopathologic examination. The diagnosis of IgG4-related disease is definitive when all three criteria are met, probable when the first and third criteria are met, and possible when the first and second criteria are met.”
IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries
Noriko Oyama-Manabe et al.
RadioGraphics 2020; 40:E29–E32 - We summarize the main characteristics of CT and/or PET/CT evaluation of IgG4-related CVD as follows: (a) For whole-body vessel assessment, use contrast-enhanced CT. Superior evaluation of aortitis is obtained in the delayed phase rather than in the early phase. (b) For follow-up, use CT to compare the changes in vessel size, such as the outer and luminal diameters and wall thickness. (c) For assessment of disease activity, evaluate the vessel maximum standardized uptake value (SUVmax) or the target-to-background ratio, rather than the blood pool SUVmax. (d) For coronary artery evaluation, ECG-gated coronary CT angiography is better than non–ECG-gated CT.”
IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries
Noriko Oyama-Manabe et al.
RadioGraphics 2020; 40:E29–E32
- “The comprehensive criteria for diagnosing IgG4- related disease in routine clinical practice: (a) characteristic diffuse or localized swelling or the presence of masses in one or more organs at clinical examination (b) elevated serum IgG4 concentration levels (≥135 mg/dL [≥1.35 g/L])at hematologic examination (c) marked lymphoplasmacytic infiltration, storiform fibrosis, and infiltration of IgG4-positive plasma cells into organs at histopathologic examination.”
IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries
Oyama-Manabe N et al.
RadioGraphics 2020; 40:E29–E32 - “Stone (6) suggested three criteria for the pathologic diagnosis of IgG4-related CVD: (a) consistency in the overall histologic findings with aortitis or periaortitis, otherwise not easily explained by another process such as atherosclerosis; (b) the presence of at least 50% IgG4-positive plasma cells; and (c) the presence of at least 50 IgG4-positive plasma cells per high-power (3400) magnification field when at least three fields are counted.”
IgG4-related Cardiovascular Disease from the Aorta to the Coronary Arteries
Oyama-Manabe N et al.
RadioGraphics 2020; 40:E29–E32
- “The features of IgG4-related CVD on contrast material–enhanced CT images include diffuse or partial arterial wall thickening (>2 mm) and homogeneous wall enhancement.We reported that 41% of patients who definitely had IgG4-related disease exhibited signs and symptoms of IgG4-related aortitis and/or periaortitis, and 80% of these patients had multiple–vascular region involvement.”
IgG4-related Cardiovascular Disease from the Aorta to the CoronaryArteries
Oyama-Manabe N et al.
RadioGraphics 2020; 40:E29–E32 - "The iliac arteries (35% of cases) and infrarenal abdominal aorta (33% of cases) were the most commonly affected in patients with IgG4-related CVD, followed by the thoracic aorta and first branches of the thoracic aorta (8% of cases), suprarenal abdominal aorta (6% of cases), and first branches of the ab- dominal aorta (5% of cases).”
IgG4-related Cardiovascular Disease from the Aorta to the CoronaryArteries
Oyama-Manabe N et al.
RadioGraphics 2020; 40:E29–E32 - “Increased wall thickness can persist for a long time after the acute phase of arterial inflammation. This characteristic limits its relevance in the early assessment of inflammation. Combined interpretation of the metabolic information from FDG PET/CT and data on vascular wall thickness from coregistered contrast-enhanced CT could be a powerful method to evaluate active inflammation in IgG4-related vasculitis.”
IgG4-related Cardiovascular Disease from the Aorta to the CoronaryArteries
Oyama-Manabe N et al.
RadioGraphics 2020; 40:E29–E32 - “We summarize the main characteristics of CT and/or PET/CT evaluation of IgG4-related CVD as follows: (a) For whole-body vessel assessment, use contrast-enhanced CT. Superior evaluation of aortitis is obtained in the delayed phase rather than in the early phase. (b) For follow-up, use CT to compare the changes in vessel size, such as the outer and luminal diameters and wall thickness. (c) For assessment of disease activity, evaluate the vessel maximum standardized uptake value (SUVmax) or the target-to-background ratio, rather than the blood pool SUVmax.”
IgG4-related Cardiovascular Disease from the Aorta to the CoronaryArteries
Oyama-Manabe N et al.
RadioGraphics 2020; 40:E29–E32 
IgG4-related Cardiovascular Disease from the Aorta to the CoronaryArteries
Oyama-Manabe N et al.
RadioGraphics 2020; 40:E29–E32- “For coronary artery evaluation, ECG-gated coronary CT angiography is better than non–ECG-gated CT. Use transaxial and reformatted images (ie, with curved planar reconstruction and in the vessel short-axis plane). (e) At follow-up exami- nations, acquisition in the same imaging plane is important for reproducible comparisons of vessel size.”
IgG4-related Cardiovascular Disease from the Aorta to the CoronaryArteries
Oyama-Manabe N et al.
RadioGraphics 2020; 40:E29–E32