Document Detail

The pathogenesis of systemic sclerosis.
MedLine Citation:
PMID:  21090968     Owner:  NLM     Status:  MEDLINE    
Systemic sclerosis (SSc), also known as scleroderma, is a rare connective tissue disease characterized by vascular and immune dysfunction, leading to fibrosis that can damage multiple organs. Its pathogenesis is complex and poorly understood. Two major clinical subtypes are the limited and diffuse forms. Research into SSc has been hampered by its rarity, its clinical heterogeneity, and the lack of mouse models that accurately recapitulate the disease. Clinical and basic studies have yielded some mechanistic clues regarding pathogenesis. Recent insights gained through the use of microarrays have revealed distinctive subsets of SSc within and beyond the limited and diffuse subsets. In this review, we discuss potential mechanisms underlying the vascular, autoimmune, and fibrotic points of dysregulation. Proper categorization of SSc patients for research studies by use of microarrays or other biomarkers is critical, as disease heterogeneity may explain some of the inconsistencies of prior studies.
Tamiko R Katsumoto; Michael L Whitfield; M Kari Connolly
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Annual review of pathology     Volume:  6     ISSN:  1553-4014     ISO Abbreviation:  Annu Rev Pathol     Publication Date:  2011  
Date Detail:
Created Date:  2011-01-25     Completed Date:  2011-04-25     Revised Date:  2012-03-14    
Medline Journal Info:
Nlm Unique ID:  101275111     Medline TA:  Annu Rev Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  509-37     Citation Subset:  IM    
Department of Medicine, University of California, San Francisco, 94143, USA.
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MeSH Terms
Scleroderma, Systemic* / etiology,  immunology,  pathology
Vascular Diseases* / etiology,  immunology,  pathology

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