Journalartikel

The Pathogenesis of Systemic Sclerosis Revisited


AutorenlisteGeyer, Matthias; Mueller-Ladner, Ulf

Jahr der Veröffentlichung2011

Seiten92-103

ZeitschriftClinical Reviews in Allergy and Immunology

Bandnummer40

Heftnummer2

ISSN1080-0549

eISSN1559-0267

DOI Linkhttps://doi.org/10.1007/s12016-009-8193-3

VerlagSpringer


Abstract
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.



Zitierstile

Harvard-ZitierstilGeyer, M. and Mueller-Ladner, U. (2011) The Pathogenesis of Systemic Sclerosis Revisited, Clinical Reviews in Allergy and Immunology, 40(2), pp. 92-103. https://doi.org/10.1007/s12016-009-8193-3

APA-ZitierstilGeyer, M., & Mueller-Ladner, U. (2011). The Pathogenesis of Systemic Sclerosis Revisited. Clinical Reviews in Allergy and Immunology. 40(2), 92-103. https://doi.org/10.1007/s12016-009-8193-3



Schlagwörter


AngiopathyBRONCHOALVEOLAR LAVAGE FLUIDCOLLAGEN GENE-EXPRESSIONDERMAL FIBROBLASTSextracellular matrixGROWTH-FACTOR-BETAIMMUNEMICROVASCULAR PERICYTESPERIPHERAL-BLOODSystemic sclerosisvasculature


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