PMID- 12055681
OWN - NLM
STAT- MEDLINE
DCOM- 20020712
LR  - 20190722
IS  - 0046-8177 (Print)
IS  - 0046-8177 (Linking)
VI  - 33
IP  - 4
DP  - 2002 Apr
TI  - Atypical manifestation of a cytoplasmic antineutrophil cytoplasmic antibody 
      (PR3-ANCA)-associated vasculitis with involvement of aortic intima and parietal 
      endocardium.
PG  - 441-5
AB  - 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.
CI  - Copyright 2002, Elsevier Science (USA). All rights reserved.
FAU - Schildhaus, Hans-Ulrich
AU  - Schildhaus HU
AD  - Institute of Pathology, University of Bonn, Germany.
FAU - Von Netzer, Barbara
AU  - Von Netzer B
FAU - Dombrowski, Frank
AU  - Dombrowski F
FAU - Pfeifer, Ulrich
AU  - Pfeifer U
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Hum Pathol
JT  - Human pathology
JID - 9421547
RN  - 0 (Antibodies, Antineutrophil Cytoplasmic)
RN  - 0 (Autoantibodies)
RN  - EC 3.4.21.- (Serine Endopeptidases)
RN  - EC 3.4.21.76 (Myeloblastin)
SB  - IM
MH  - Antibodies, Antineutrophil Cytoplasmic/*immunology
MH  - Aorta/*immunology/pathology
MH  - Autoantibodies/analysis
MH  - Endocardium/*immunology/pathology
MH  - Heart Atria/pathology
MH  - Heart Ventricles/pathology
MH  - Humans
MH  - Immunohistochemistry
MH  - Male
MH  - Microscopy, Electron
MH  - Middle Aged
MH  - Myeloblastin
MH  - Serine Endopeptidases/immunology
MH  - Vasculitis/*immunology/pathology
EDAT- 2002/06/11 10:00
MHDA- 2002/07/13 10:01
CRDT- 2002/06/11 10:00
PHST- 2002/06/11 10:00 [pubmed]
PHST- 2002/07/13 10:01 [medline]
PHST- 2002/06/11 10:00 [entrez]
AID - S0046817702000102 [pii]
AID - 10.1053/hupa.2002.124331 [doi]
PST - ppublish
SO  - Hum Pathol. 2002 Apr;33(4):441-5. doi: 10.1053/hupa.2002.124331.