Background Granulomatosis with polyangiitis (GPA, Wegener's granulomatosis) is an antineutrophil cytoplasm antibody (ANCA)–associated small-vessel vasculitis of unknown etiology, which is characterized by ear, nose, and throat (ENT); lung; and kidney involvement. Cardiac manifestations are generally considered rare in GPA patients, but they may be life-threatening.
Objectives The aim of this study was to determine the frequencies and types of cardiac manifestations in patients with granulomatosis with polyangiitis (Wegener's, GPA) at diagnosis.
Methods This retrospective analysis was conducted on the cardiac manifestations of GPA patients entered into the French Vasculitis Study Group database between July 1955 and September 2014.
Results Among the 671 identified patients, 39 (5.8%) had cardiac manifestations at diagnosis. Mean age at diagnosis was 51.1±20.1 years, with no remarkable sex predominance (male/female sex ratio, 1.4) and the median time from symptom onset to diagnosis was 2 months. The most frequent extracardiac manifestations at diagnosis were ENT (30, (77%)), pulmonary (29, (76.3%)) and renal (28, (71.8)) manifestations. The 3 most common recorded cardiac manifestations were pericarditis (16, (41%)), myocarditis (11, (28.2%)), and coronary vasculitis (5, (12.8%)). Aortic valvulopathy (4, (10.3%)) and arrhythmias (4, (10.3%)) were also common feature. Mean BVAS at diagnosis was 21.9±9.6. 25 (64.1%) GPA patients were PR3-ANCA positive. Median serum creatinine and CRP were 147 μmol/L (IQR: 82.5-283.5) and 112 mg/l (IQR: 14-202) respectively. Median follow-up was 37 months (IQR: 18-86). 15 (38.5%) experienced at least one relapse. 7 cardiac relapse occurred in 4 GPA patients, mostly pericarditis (5/7). 13 (33.3%) GPA patients died during follow-up.
Conclusions Cardiac manifestations at diagnosis were rare in GPA. They occurred in patients with a severe onset of the disease and close treatment and monitoring is critical.
Disclosure of Interest None declared