Document Detail


Pulmonary veno-occlusive disease as a primary cause of pulmonary hypertension in a patient with mixed connective tissue disease.
MedLine Citation:
PMID:  17520258     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Mixed connective tissue disease (MCTD) is a systemic disease seen in a group of patients with overlapping clinical features of lupus, scleroderma, polymyositis, and rheumatoid arthritis. A defining feature of MCTD is the presence of antibodies against the U1-ribonucleoprotein (U1-RNP) complex. Pulmonary hypertension is the major cause of death in MCTD. We report an autopsy case of MCTD with pulmonary hypertension. The U1-RNP antibody of this patient was 171.9 U (normal < 25.0 U). The immediate cause of death was attributed to acute pulmonary embolism at left lower lobe. A severe vasculopathy characterized by fibrotic occlusion of small veins and venules, associated with prominent capillary congestion, was consistent with pulmonary veno-occlusive disease (PVOD). This is the first case reported in which PVOD is the primary cause of pulmonary hypertension in MCTD.
Authors:
Lizhi Zhang; Daniel Visscher; Charanjit Rihal; Marie-Christine Aubry
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2007-05-23
Journal Detail:
Title:  Rheumatology international     Volume:  27     ISSN:  0172-8172     ISO Abbreviation:  Rheumatol. Int.     Publication Date:  2007 Oct 
Date Detail:
Created Date:  2007-09-06     Completed Date:  2008-01-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8206885     Medline TA:  Rheumatol Int     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1163-5     Citation Subset:  IM    
Affiliation:
Department of Pathology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA.
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MeSH Terms
Descriptor/Qualifier:
Antibodies / blood
Fatal Outcome
Female
Humans
Hypertension, Pulmonary / etiology*,  immunology,  pathology
Middle Aged
Mixed Connective Tissue Disease / complications*,  immunology,  pathology
Pulmonary Veins / pathology*,  physiopathology
Pulmonary Veno-Occlusive Disease / complications*,  immunology,  pathology
Ribonucleoprotein, U1 Small Nuclear / immunology
Chemical
Reg. No./Substance:
0/Antibodies; 0/Ribonucleoprotein, U1 Small Nuclear

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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