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

Abstract

The prevalence and extent of immunoglobulin G4 (IgG4)-positive cell infiltration were investigated in 282 surgical samples of aortic wall and aortic valve. Tissue infiltration of IgG4-positive cells was observed in 24 (17.3%) of 139 aortic valve samples and 46 (32%) of 143 aortic wall samples, and the condition of IgG4positive cell infiltration > 30/hpf together with IgG4/CD138 ratio > 40% was observed in 2 (1.4%) of aortic valve samples and 14 (9.8%) of aortic wall samples. Among 275 patients, preoperative serum IgG4 level was available in 48 patients (50 samples), and it was > 135 mg/dL in only one patient. Of these 48 patients with serum IgG4 measurement, 29 patients had aortic valve stenosis and 12 had aortic aneurysm. Compared with 23 aortic stenosis patients without tissue infiltration of IgG4-positive cells in the aortic valve, six patients with IgG 4-positive cell infiltration had a more prevalent smoking history (26% versus 83%) and borderline significantly higher serum IgG4 (median, 24.5 mg/dL versus 55.5 mg/dL), although either preoperative peak pressure gradient between left ventriculum and aorta or aortic valve area did not differ significantly between groups. Compared with six aortic aneurysm patients without tissue infiltration of IgG4-positive cells in the aortic wall, six patients with IgG4-positive cell infiltration had borderline significantly higher serum IgG4 (median, 28.9 mg/dL versus 68.2 mg/dL). The current study showed that tissue IgG4-positive infiltration is not a rare occurrence in the aortic stenosis and aortic aneurysm. Clinical significance of tissue IgG4-postive cell infiltration in these patients requires further investigation.

Vascular Involvement of Immunoglobulin G4-Related Disease: A Case Report

“…A decade ago, the first documented cases of vascular involvement in IgG4 disease emerged, with subsequent observations indicating a high prevalence of involvement in the abdominal and thoracic aorta, manifested as aortitis or periaortitis [ 3 ]. However, the term periarteritis was later emphasized to describe medium-large sized arteries affected by arterial wall thickening [ 4 ].…”

Vasculitis beyond aortitis in IgG4-related disease (IgG4-RD): case report and review of the literature

“…The term "IgG4-RD periarteritis" has been adopted to describe this situation. Noteworthy, other parts of cardiovascular system (e.g., heart valves) are involved [7].…”

Classification of IgG4‐Related Disease: A Medical Marvel of Our Time

“…Reasonable precaution will be required, as the criteria necessitate judgment and diligence when used for any reason other than the assembly of a cohort for study and research. Unusual sites of infiltration have been reported, such as hypophysitis , thymus , temporal lobe , true ocular infiltrate , or aortic root involvement . Readers are reminded that classification criteria do not comprise the complete list of disease manifestations.…”