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01 April 2019 : Case report  Denmark

Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation

Challenging differential diagnosis, Rare disease

Thomas Jeremy Guterbaum1CEF*, Mirza Husic2B, Anne Voss3DE, Jordi Sanchez Dahl1DEF

DOI: 10.12659/AJCR.912693

Am J Case Rep 2019; 20:423-429

Abstract

BACKGROUND: Granulomatosis with polyangiitis (GPA)/Wegener’s granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/Churg-Strauss’ syndrome (CSS) are ANCA (antineutrophil cytoplasmic antibodies) associated vasculitides that can affect the heart, predominantly the myocardium. Valvular affection is rare and is described anecdotally. The purpose of this case report was to present aortic valve affection of an ANCA positive vasculitis.

CASE REPORT: We present the case with a 56-year-old male diagnosed with ANCA associated vasculitis, who began experiencing respiratory symptoms primarily thought to be respiratory tract affection. These symptoms worsened, and an echocardiography revealed heart failure with decreased left ventricular ejections fraction (EF=30–35%) and a severe insufficiency of the aortic valve. The patient underwent aortic valve replacement with symptomatic relief. Pathological examination of aortic valve resectates revealed inflammation and thickening of the aortic cusps.

CONCLUSIONS: Patients with ANCA associated vasculitis can rarely present with valvular inflammation causing severe regurgitation. The aortic valve can be involved, although cases have also described mitral valve involvement and both valves simultaneously. In patients with ANCA associated vasculitis a severe worsening of dyspnea can be caused by exacerbation of pulmonary involvement, but severe valvular disease should also be considered.

Keywords: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Aortic Valve Insufficiency, Heart Failure, Middle Aged, Stroke Volume, Ventricular Dysfunction, Left

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923