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Review
. 2018 Jan-Mar;40(1):77-81.
doi: 10.1590/2175-8239-JBN-3807. Epub 2018 May 7.

Complement activation in atypical hemolytic uremic syndrome and scleroderma renal crisis: a critical analysis of pathophysiology

[Article in English, Portuguese]
Roman Zuckerman  1 , Arif Asif  1 , Eric J Costanzo  1 , Tushar Vachharajani  2
Affiliations
Review

Complement activation in atypical hemolytic uremic syndrome and scleroderma renal crisis: a critical analysis of pathophysiology

[Article in English, Portuguese]
Roman Zuckerman et al. J Bras Nefrol. 2018 Jan-Mar.

Abstract
in English, Portuguese

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.

A esclerodermia é uma doença autoimune que afeta múltiplos sistemas. Embora os mecanismos fisiopatológicos que regem o desenvolvimento da esclerodermia sejam relativamente pouco compreendidos, os avanços em nossa compreensão do sistema do complemento estão esclarecendo o papel das vias do complemento no desenvolvimento da síndrome urêmica hemolítica atípica e da crise renal da esclerodermia. As abundantes semelhanças em sua apresentação, bem como o curso clínico, estão aumentando a possibilidade de uma patogênese subjacente comum. Relatórios recentes estão enfatizando que as vias de complemento parecem ser o link unificador. Este artigo analisa o papel do sistema do complemento no desenvolvimento da síndrome urêmica hemolítica atípica e da crise renal na esclerodermia, e exige maior conscientização para com o desenvolvimento da angiopatia trombótica em pacientes com esclerodermia.

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Conflict of interest statement

CONFLICTS OF INTERES

None of the authors has any disclosure except for AA who has served on speaker bureau for Alexion Pharmaceuticals. However, no grant/funding was obtained for this paper.

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References

    1. LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Jr, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15:202–205. - PubMed
    1. Wollheim FA. Rheumatology. Vol. 44. Oxford: 2005. Classification of systemic sclerosis. Visions and reality; pp. 1212–1216. - PubMed
    1. Steen VD. Scleroderma renal crisis. Rheum Dis Clin North Am. 2003;29:315–333. - PubMed
    1. Penn H, Howie AJ, Kingdon EJ, Bunn CC, Stratton RJ, Black CM, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. 2007;100:485–494. - PubMed
    1. Hudson M, Baron M, Tatibouet S, Furst DE, Khanna D, International Scleroderma Renal Crisis Study Investigators Exposure to ACE inhibitors prior to the onset of scleroderma renal crisis-results from the International Scleroderma Renal Crisis Survey. Semin Arthritis Rheum. 2014;43:666–672. - PubMed

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