IgG4‐related inflammatory aneurysm of the aortic arch

Abstract

IgG4-related sclerosing disease can occur in the cardiovascular system and some inflammatory abdominal aortic aneurysms have been shown to belong to IgG4-related sclerosing disease. Herein is reported a case of IgG4-related inflammatory aortic aneurysm of the aortic arch. A 71-year-old Japanese man was found to have an aneurysm of the aortic arch with maximum dimension of 5.5 cm. The surgically resected aneurysm wall had conspicuous fibrosclerotic changes, dense lymphoplasmacytic infiltration and occasional obliterative phlebitis in the adventitia; the thickness of the adventitia was 6.5 mm. Immunohistochemistry indicated numerous IgG4-positive plasma cell infiltrates; 84% of the IgG-bearing cells were IgG4 positive. The diagnosis of IgG4-related inflammatory aortic aneurysm of the aortic arch was made. Although previously reported IgG4-related inflammatory aortic aneurysms were confined to the abdominal aorta, the present case report demonstrates that IgG4-related inflammatory aortic aneurysm can occur in the aortic arch, thereby extending the spectrum of IgG4-related periaortitis. Further studies are needed to clarify the spectrum of IgG4-related sclerosing disease in the cardiovascular system.

Sudden Cardiac Death Due to Coronary Artery Involvement by IgG4-Related Disease: A Rare, Serious Complication of a Rare Disease

“…Inflammatory aortic aneurysms are usually seen within the infrarenal abdominal aorta. 9 A subset of cases of inflammatory abdominal aortic aneurysm are due to IgG4-RD, although concomitant atherosclerotic changes can be seen in the media and intima. 5 These cases also show fibrous adhesions extending into the surrounding soft tissue.…”

“…In general, inflammatory aortic aneurysm involving the ascending aorta or aortic arch is exceedingly rare, but IgG4-related inflammatory aneurysm of the aortic arch has been reported. 9 IgG4-RD may also manifest as an inflammatory retroperitoneal lesion resulting from involvement of the abdominal aorta, kidneys, or ureters. 8 In summary, all of these findings led Stone 8 to conclude that adventitial involvement is a feature of IgG4-related aortic lesions, while Kasashima and Zen 5 emphasized that stricture and occlusive changes are not commonly seen.…”

IgG4-related Inflammatory Abdominal Aortic Aneurysm, Spectrum of IgG4-related Chronic Periaortitis

“…40) IgG4-related thoracic aortic lesions often represent aneurismal cases, represented by ITAA. 39,40) Interestingly, several cases of lymphoplasmacytic aortitis without dilation of the aorta were included in IgG4-related thoracic aortic lesions. 41,42) In the aorta, an aneurysm may be caused by destruction of the vascular wall structure by severe inflammation.…”

“…However, very few cases of ITAA have been reported compared to IAAA. 38,39) Since diverse lesions, such as aor tic dissection and aor titis syndromes (Takayasu disease and giant cell vasculitis), frequently occur in the thoracic aorta, ITAA has been a vague entity and often classed with other special types of thoracic aortitis.…”

IgG4-Related Cardiovascular Disorders

“…5) In 2001, Type 1 AIP with elevated serum IgG4 level and abundant IgG4-positive lymphoplasmacyte infiltration in the pancreas was reported and the concept of IgG4-related disease was proposed. 6) Then, it was gradually discovered that IgG4-related disease involves inflammation and sclerosing in many other organs, 7) especially exocrine organs: 8) bile ducts, 9) lacrimal and salivary glands, 10) retroperitoneum, 11) urinary organs, [12][13][14] pituitary gland, 15) prostate, 16) arteries, [17][18][19][20] veins, [21][22][23] heart, [24][25][26] lung, 25,27,28) eye, 24,29) mesenterium, 30) mammary gland, 31) central nervous system, 32) esophagus, 33) stomach, 34) liver, 35) gastrointestinal tract, 36) thyroid, 37,38) nose, 39) blood, 40,41) skin, 42) and pseudotumors in various organs 9,14,25,[43][44]…”