title: A case of aortic and mitral valve involvement in granulomatosis with polyangiitis.
abstract: Granulomatosis with polyangiitis (GPA) (Wegener's) is a necrotizing systemic vasculitis of the small-sized blood vessels, affecting kidneys, lungs, upper respiratory tract and skin. Cardiac valvular involvement is an uncommon manifestation of GPA. We report the case of a 60-year-old woman with arthritis and lung nodules due to GPA without antineutrophil cytoplasmic antibodies (ANCA) at time of diagnosis. Remission was obtained with cyclophosphamide and corticosteroid. Azathioprine was then prescribed for 2years. Four years later, she developed severe inflammatory aortic and mitral valvular involvement characterized by GPA typical histopathological valvular lesions. Search for ANCA was positive at this time (anti-myeloperoxidase). Cardiac valvular involvement is a rare and potentially fatal complication of GPA and may misleadingly suggest infectious endocarditis. A review of literature revealed few cases of histologically well-documented cardiac valvular involvement in GPA. Pathologists should be aware of valvular heart diseases in GPA, which usually comprise valvular necrotic lesions without any microbial agents.
authors.affiliation: Department of Internal Medicine, University Hospital of Nantes, France. Electronic address: olivier.espitia@chu-nantes.fr.
authors.forename: Olivier
authors.initials: O
authors.lastname: Espitia
authors.affiliation: Department of Pathology, University Hospital of Nantes, France.
authors.forename: Laure
authors.initials: L
authors.lastname: Droy
authors.affiliation: Department of Cardiology, University Hospital of Nantes, France.
authors.forename: Sabine
authors.initials: S
authors.lastname: Pattier
authors.affiliation: Department of Pulmonary Medicine, University Hospital of Nantes, France.
authors.forename: Frédérique
authors.initials: F
authors.lastname: Naudin
authors.affiliation: Department of Thoracic and Cardio-vascular Surgery, University Hospital of Nantes, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Mugniot
authors.affiliation: Department of Pulmonary Medicine, University Hospital of Nantes, France.
authors.forename: Arnaud
authors.initials: A
authors.lastname: Cavailles
authors.affiliation: Department of Internal Medicine, University Hospital of Nantes, France.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.affiliation: Department of Pathology, Hôpital Européen Georges Pompidou, Paris, France.
authors.forename: Patrick
authors.initials: P
authors.lastname: Bruneval
authors.affiliation: Department of Internal Medicine, University Hospital of Nantes, France.
authors.forename: Christian
authors.initials: C
authors.lastname: Agard
authors.affiliation: Department of Pathology, University Hospital of Nantes, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Toquet
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001021:Aortic Valve / Q000473:pathology*; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000276:immunology / Q000473:pathology*; D006349:Heart Valve Diseases / Q000276:immunology / Q000473:pathology*; D006801:Humans; D008875:Middle Aged; D008943:Mitral Valve / Q000473:pathology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic
keywords: Epithelioid granuloma; Granulomatosis with polyangiitis; Polymorphous microabscesses; Valve disease; Wegener’s granulomatosis