A Case of Aortic Stenosis with Serum IgG4 Elevation, and IgG4-Positive Plasmacytic Infiltration in the Aortic Valve, Epicardium, and Aortic Adventitia

Abstract

A 74-year-old man was admitted for preoperative screening of aortic stenosis. Five months before this admission, he was found to have elevated serum immunoglobulin G4 (IgG4; 2,010 mg/dL). Computed tomography (CT) showed a soft tissue mass surrounding the abdominal aorta, suggestive of IgG4-related periaortitis. CT coronary angiography showed perivascular thickening of the right coronary artery, and subsequent coronary angiography showed a multi-vessel disease. The patient underwent aortic valve replacement and coronary bypass surgery. Immunohistochemical analysis showed IgG4-positive plasmacytic infiltration in specimens from the aortic valve, epicardium, and aortic adventitia, suggestive of the possible role of IgG4-related immune inflammation for the pathogenesis.

IgG4-Positive Plasmacytic Infiltration in Aortic Wall and Aortic Valve Surgical Samples and Its Relation to Preoperative Serum IgG4 Levels

“…In Maleszewski, et al's report, one patient with dense IgG4-positive cell infiltration in the aortic valve showed normal range serum IgG4 (90 mg/dL), whereas the other patient, who also had noncardiac organ involvement (autoimmune pancreatitis), had markedly elevated serum IgG4 (> 1000 mg/dL). 13) It is possible that serum IgG4 level may not be elevated when aortic valve is the only region where IgG4-positive cell infiltration is present 8) or the extent of infiltration is only minor. Another important finding of the current study was that IgG4-positive cell infiltration of > 10/hpf with an IgG4/CD138 ratio of > 40% was observed in 19 (13.2%) of 143 aortic wall specimens.…”

“…We previously demonstrated in a preliminary study that tissue infiltration of IgG4-positive cell may be observed with the frequency of more than 10% in surgically resected samples of the aortic wall and aortic valve. 7,8) The aim of the current study is to reassess IgG4-positive cell infiltration in aortic wall and aortic valve samples by a larger sample size and to examine whether preoperative serum IgG4 levels, when available, were associated with IgG4-positive cell infiltration in these tissues.…”

IgG4-related periaortitis/periarteritis: An under-recognized condition that is potentially life-threatening

“…In general, patients with IgG4-related disease have been frequently observed to have a history of malignancy preceding the clinical onset of IgG4-related disease [15]. In some studies, a history of malignancies has also been among patients with IgG4-related periaortitis/periarteritis [16,17]; however, whether the prevalence of malignancy differs between patients with vascular lesions and those with nonvascular lesions remains unclear.…”

Immunoglobulin G4‐related disease associated with asymptomatic aortic stenosis and diagnosed based on skin lesions

“…Recent studies have shown that IgG4-RD may target parts of the cardiovascular system, including the aorta, peripheral arteries and pericardium. 3,4 In addition, immunohistochemical analysis of tissue specimens has indicated that infiltration of IgG4positive plasma cells may not be a rare occurrence in various cardiac and arterial disorders. 4 However, only pathological findings can confirm whether AS is caused by IgG4-RD if heart valve prosthesis surgery or another procedure is performed.…”