Coronary (peri)-arteritis in patients with IgG4-related disease: A case series from the Central Anatolia Region of Turkey

Objective Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibro-inflammatory disease which affects many systems, as well as the cardiovascular system. Identifying the coronary involvement like periaortitis, coronary periarteritis and pericarditis is important, as they often cause unfavorable outcomes. Methods Eighty-one patients with IgG4-RD were retrospectively evaluated for symptomatic coronary artery involvement from Hacettepe University Vasculitis Research Center (HUVAC) database. The demographic, laboratory, radiologic and clinical characteristics of the patients were assessed. Results Among 81 patients with IgG4-RD, 6 patients (M/F:5/1) had coronary artery involvement. The patients' median age was 57 and serum IgG4 levels were above normal except for one case. All patients with coronary arteritis revealed an increased coronary vessel wall thickening and stenotic lesions. The coronary aneurysm and pericarditis were observed in half of the patients. Immunosuppressive treatments were given to all the patients and most of them followed in stable condition. Conclusion Coronary arteritis is a rare but notable manifestation of IgG4-RD. Although coronary periarteritis can cause significant morbidity and mortality, it seems better results can be achieved with early diagnosis and treatment.

Automated summary

IgG4-related disease is a fibro-inflammatory disease affecting multiple systems, including the cardiovascular system. Coronary artery involvement, although rare, can lead to significant morbidity and mortality. Early diagnosis and treatment appear to result in better outcomes for patients with coronary arteritis.