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The pathogenesis of systemic sclerosis revisited.
MedLine Citation:
PMID:  20087791     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Although infectiological stimuli, environmental factors and genotypic features are known to contribute to the initiation and perpetuation of systemic sclerosis (SSc), its etiology still remains to be enigmatic, and less elusive insights are to be achieved by ongoing and future investigations. Being characterized, however, as chronic autoimmune disease with excessive collagen accumulation in skin, synovia and visceral organs such as lung, heart, and digestive tract along with obliterating angiopathy, the pathophysiology of SSc can be summarized as being based on imbalances of the cellular and humoral immune system, vascular dysfunction and activation of resident connective tissue cells. A complex interplay between these major components manages to establish and maintain the inability of the vasculature to adequately react to the need for dilatation, constriction and growth of new vessels, to cause the increased deposition of extracellular matrix constituents as well as to facilitate immunological disarrangement. Despite parallels to the chicken and egg causality dilemma, all of these account for what later clinicians observe in patients suffering from Raynaud's phenomenon, digital ulcers, sclerodactyly, rigidity of the face, microstomia, sicca syndrome, dyspnea, dry cough, pulmonary hypertension, palpitations, syncopes, renal insufficiency, dysphagia, gastroesophageal reflux, dyspepsia, generalized arthralgias, but also dyspareunia, or erectile dysfunction.
Authors:
Matthias Geyer; Ulf Müller-Ladner
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Clinical reviews in allergy & immunology     Volume:  40     ISSN:  1559-0267     ISO Abbreviation:  Clin Rev Allergy Immunol     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-02-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9504368     Medline TA:  Clin Rev Allergy Immunol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  92-103     Citation Subset:  IM    
Affiliation:
Department of Rheumatology and Clinical Immunology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Benekestr. 2-8, 61231, Bad Nauheim, Germany.
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