title: Atypical manifestation of a cytoplasmic antineutrophil cytoplasmic antibody (PR3-ANCA)-associated vasculitis with involvement of aortic intima and parietal endocardium.
abstract: The traditional classification of vasculitis, based on the size of affected vessels, has meanwhile been extended by using antineutrophil cytoplasmic antibodies (ANCAs) as seromarkers in the differential diagnosis of different types of vasculitis. We report an autopsy case of fulminant generalized vasculitis positive for C-ANCA (1:320) and anti-proteinase 3 (PR3) antibodies (>100 U/mL) in a 63-year-old man. The unusually broad histologic spectrum included periarteritis nodosa-like lesions in medium-sized vessels and leucocytoclastic vasculitis in small vessels, as well as capillaritis. In addition, the left atrial and ventricular endocardium and the intima of the aorta thoracalis were patchily involved in the inflammatory process. Glomerulonephritis and/or immune complexes were not detectable by electron microscopy or immunohistochemistry. To the best of our knowledge, involvement of the aortic intima ("intimitis") and the parietal endocardium has not been described in PR3-ANCA-positive vasculitis to date.
authors.affiliation: Institute of Pathology, University of Bonn, Germany.
authors.forename: Hans-Ulrich
authors.initials: HU
authors.lastname: Schildhaus
authors.forename: Barbara
authors.initials: B
authors.lastname: Von Netzer
authors.forename: Frank
authors.initials: F
authors.lastname: Dombrowski
authors.forename: Ulrich
authors.initials: U
authors.lastname: Pfeifer
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001011:Aorta / Q000276:immunology* / Q000473:pathology; D001323:Autoantibodies / Q000032:analysis; D004699:Endocardium / Q000276:immunology* / Q000473:pathology; D006325:Heart Atria / Q000473:pathology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D053146:Myeloblastin; D012697:Serine Endopeptidases / Q000276:immunology; D014657:Vasculitis / Q000276:immunology* / Q000473:pathology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D012697:Serine Endopeptidases; D053146:Myeloblastin