Affiliation:
1. Jersey Shore University Medical Center, USA
2. W.G. (Bill) Hefner VA Medical Cente, USA
Abstract
ABSTRACT Scleroderma is an autoimmune disease that affects multiple systems. While pathophysiologic mechanisms governing the development of scleroderma are relatively poorly understood, advances in our understanding of the complement system are clarifying the role of complement pathways in the development of atypical hemolytic uremic syndrome and scleroderma renal crisis. The abundant similarities in their presentation as well as the clinical course are raising the possibility of a common underlying pathogenesis. Recent reports are emphasizing that complement pathways appear to be the unifying link. This article reviews the role of complement system in the development of atypical hemolytic uremic syndrome and scleroderma renal crisis, and calls for heightened awareness to the development of thrombotic angiopathy in patients with scleroderma.
Reference62 articles.
1. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis;LeRoy EC;J Rheumatol,1988
2. Classification of systemic sclerosis. Visions and reality;Wollheim FA;Rheumatology,2005
3. Scleroderma renal crisis;Steen VD;Rheum Dis Clin North Am,2003
4. Scleroderma renal crisis: patient characteristics and long-term outcomes;Penn H;QJM,2007
5. Exposure to ACE inhibitors prior to the onset of scleroderma renal crisis-results from the International Scleroderma Renal Crisis Survey;Hudson M;Semin Arthritis Rheum,2014
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