Solitary recurrence of IgG4-related giant coronary aneurysm: Case report and review of the literature focusing on treatment strategies and complications

Abstract

Coronary periarteritis is a dangerous manifestation of IgG4-related disease, because it forms coronary artery aneurysms, which may cause sudden cardiac death. We report the case of a 78-year-old woman with IgG4-related coronary periarteritis and a coronary aneurysm, which showed progressive enlargement despite maintenance therapy for Type 1 autoimmune pancreatitis. This case was unique, in that coronary periarteritis was the only active lesion that recurred. Low-dose glucocorticoids suppressed the progression of periarterial lesions but led to rapid thinning of the aneurysmal wall and an increase in the size of mural thrombi, which pose a risk of myocardial infarction. Our systematic literature review including 98 cases of 86 articles was performed to examine its treatment strategies and complications. Among the cases in which the effect of immunosuppressive therapy could be followed radiologically, 33 of 37 (89.1%) cases showed improvement in wall thickening/periarterial soft tissue, while 6 of 13 (46.2%) showed worsening increase in the outer diameter of the coronary aneurysms. We propose a draft treatment algorithm and suggest that immunosuppressive therapy for IgG4-related coronary periarteritis with coronary aneurysms should be conducted only after the therapeutic benefit has been determined to outweigh the risks. Because coronary periarteritis can occur without other organ involvement, as in our case, all cases of IgG4-related disease require careful monitoring of coronary artery lesions.

Validation of the Diagnostic Criteria for IgG4-Related Periaortitis/Periarteritis and Retroperitoneal Fibrosis (IgG4PA/RPF) 2018, and Proposal of a Revised 2023 Version for IgG4-Related Cardiovascular/Retroperitoneal Disease

“…Matsumoto et al 25 reported many cases with coronary periarteritis and/or aneurysm. Literature reviews by Akiyama et al 26 and Kawahara et al 27 reported that patients with IgG4-related coronary artery involvement often exhibited stenotic lesions (54-67% of patients), aneurysmal lesions (42-43%), and diffuse wall thickening (92-96%). Glucocorticoid treatment, either alone or in combination with percutaneous coronary intervention or cardiac surgery, has proven effective in managing most cases of IgG4-related coronary artery involvement.…”

“…Glucocorticoid treatment, either alone or in combination with percutaneous coronary intervention or cardiac surgery, has proven effective in managing most cases of IgG4-related coronary artery involvement. However, 46% of cases with coronary artery aneurysm showed worsening of the outer diameter of the coronary aneurysms, 27 underscoring the importance of establishing an appropriate diagnosis and treatment strategy. In the present study, the revised diagnostic criteria for IgG4-related cardiovascular/retroperitoneal disease demonstrated an acceptable sensitivity and specificity for the diagnosis of patients with coronary artery lesions.…”