title: Valvular involvement in ANCA-associated systemic vasculitis: a case report and literature review.
abstract: BACKGROUND
  Antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitides have a variety of presentations, but cardiac valvular involvement is rarely diagnosed and its management is not established.
  
  
  METHODS
  We report the case of a 44 year old man who presented with an ANCA-associated systemic vasculitis and aortic regurgitation of unusual mechanism. Transthoracic and transesophageal echocardiography disclosed septal hypertrophy preventing a complete closure of the aortic valve and thus responsible for a massive aortic regurgitation. After 4 months of immunosuppressive therapy, the valve lesion did not subside and the patient had to undergo aortic valve replacement. This report also reviews the 20 cases of systemic ANCA-associated vasculitis with endocardial valvular involvement previously reported in the English language medical literature.
  
  
  CONCLUSIONS
  Valvular involvement in ANCA-associated systemic vasculitides is rarely reported. Most of these lesions are due to Wegener's granulomatosis and half are present when the diagnosis of vasculitis is made. The valvular lesion is usually isolated, aortic regurgitation being the most frequent type, and often requires valve replacement in the months that follow it's discovery.
authors.affiliation: Assistance Publique Hôpitaux de Paris (AP-HP), department of internal medicine, Ambroise Paré Hospital, 9, avenue Charles-de-Gaulle, 92100, Boulogne Billancourt, France.
authors.forename: Chloé
authors.initials: C
authors.lastname: Lacoste
authors.forename: Nicolas
authors.initials: N
authors.lastname: Mansencal
authors.forename: Mona
authors.initials: M
authors.lastname: Ben M'rad
authors.forename: Catherine
authors.initials: C
authors.lastname: Goulon-Goeau
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Thomas
authors.initials: T
authors.lastname: Hanslik
mesh_terms: D000328:Adult; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000503:physiopathology*; D001021:Aortic Valve / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000503:physiopathology; D013504:Cardiovascular Surgical Procedures / Q000379:methods; D017548:Echocardiography, Transesophageal; D006801:Humans; D008297:Male; D016896:Treatment Outcome