ANCA-Associated Vasculitides Valvular Impairment: Multicenter Retrospective Study and Systematic Review of the Literature.

OBJECTIVE: While myocardial impairment is a predictor of poor prognosis in ANCA-associated vasculitides (AAV), little is known about valvular involvement. This study aims at describing the clinical presentation, management and outcome of endocarditis associated with AAV.

METHODS: We conducted a multicenter retrospective study in centers affiliated with the French Vasculitis Study Group. We included patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic granulomatosis with polyangiitis (EGPA) with endocardial impairment. A systematic review was then performed through PubMed, Embase and Cochrane library up to September 2020.

RESULTS: The retrospective cohort included ten patients (91%) with GPA and one (9%) with MPA. Clinical presentation included acute valvular insufficiency (n=7, 64%), cardiac failure (n=3, 27%), dyspnea (n=3, 27%), or no symptom (n=2, 18%). The aortic valve was the most frequently affected (n=8/10, 80%), and vegetations were noted in 4/10 patients (40%). Six patients (55%) underwent surgical valvular replacement. No death from endocarditis was reported. The systematic review retrieved 42 patients from 40 references: 30 (71%) had GPA, 21 (50%) presented with vegetations, the aortic valve (n = 26, 62%) was mostly involved. Valvular replacement was required in 20 cases (48%) and 5 patients (13%) died from the endocarditic impairment.

CONCLUSION: Endocarditis is a rare manifestation of AAV but might be underdiagnosed. Acute valvular insufficiency may lead to urgent surgery. Implementing transthoracic echocardiography in standard assessment at baseline and follow-up of AAV might reduce the delay to diagnosis and allow earlier specific immunosuppressive treatment before surgery is needed.