Document Detail


Churg-Strauss syndrome: evidence for disease subtypes?
MedLine Citation:
PMID:  19851111     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PURPOSE OF REVIEW: Churg-Strauss syndrome (CSS) is a rare systemic small-vessel necrotizing vasculitis. Its main clinical characteristics, some potentially life-threatening, are now well known, as are its usual successive phases, from allergic rhinitis to asthma, and finally vasculitis. Conversely, physiopathogenetic mechanisms are not completely elucidated and clearly multiple, thereby suggesting the existence of different disease subtypes. RECENT FINDINGS: Almost 40% of CSS patients have circulating antineutrophil cytoplasm autoantibodies (ANCAs), mostly directed against myeloperoxidase. ANCA-positive patients suffer more frequently from renal disease, peripheral nervous system involvement and/or alveolar hemorrhage, whereas frequent cardiac involvement, lung infiltrates and/or systemic manifestations are more common in those who are ANCA-negative. However, their respective global outcomes do not clearly differ. Patients might also be categorized according to other, more subtle clinical, radiological and/or biological parameters, for example, cardiac magnetic resonance imaging abnormalities or genetic background. SUMMARY: Because of its practical and therapeutic repercussions, the priority remains the prompt, relatively easy identification of the most severely affected patients at CSS diagnosis, before searching for and trying to classify subsets. Large, collaborative studies are needed to determine whether other subgroups might be associated with outcomes and warrant different, and possibly new, therapeutic strategies.
Authors:
Christian Pagnoux; Lo?c Guillevin
Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Current opinion in rheumatology     Volume:  22     ISSN:  1531-6963     ISO Abbreviation:  Curr Opin Rheumatol     Publication Date:  2010 Jan 
Date Detail:
Created Date:  2009-11-25     Completed Date:  2010-02-16     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9000851     Medline TA:  Curr Opin Rheumatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  21-8     Citation Subset:  IM    
Affiliation:
Department of Internal Medicine, French Vasculitis Study Group, National Referral Center for Necrotizing Vasculitides and Systemic Scleroderma, H?pital Cochin, Assistance Publique-H?pitaux de Paris, Universit? Paris-Descartes, Paris, France. christian.pagnoux@cch.aphp.fr
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MeSH Terms
Descriptor/Qualifier:
Antibodies, Antineutrophil Cytoplasmic / metabolism*
Autoimmunity / genetics,  physiology
Biological Markers
Churg-Strauss Syndrome / classification*,  diagnosis,  physiopathology*
Disease Progression
Early Diagnosis
Humans
Outcome Assessment (Health Care) / standards
Peroxidase / immunology
Chemical
Reg. No./Substance:
0/Antibodies, Antineutrophil Cytoplasmic; 0/Biological Markers; EC 1.11.1.7/Peroxidase

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


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