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International Journal of Rheumatology
Volume 2010 (2010), Article ID 543704, 7 pages
Review Article

Scleroderma Renal Crisis: A Pathology Perspective

1Department of Pathology, University of Pittsburgh Medical Center, A614 Scaife Hall, 200 Lothrop Street, Pittsburgh, PA 15213, USA
2Division of Rheumatology and Clinical Immunology, University of Pittsburgh Medical Center, 3500 Terrace Street, BST, S7, Pittsburgh, PA 15261, USA

Received 14 May 2010; Accepted 28 June 2010

Academic Editor: Lorinda Chung

Copyright © 2010 Ibrahim Batal et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Scleroderma renal crisis (SRC) is an infrequent but serious complication of systemic sclerosis (SSc). It is associated with increased vascular permeability, activation of coagulation cascade, and renin secretion, which may lead to the acute renal failure typically associated with accelerated hypertension. The histologic picture of SRC is that of a thrombotic microangiopathy process with prominent small vessel involvement manifesting as myxoid intimal changes, thrombi, onion skin lesions, and/or fibrointimal sclerosis. Renal biopsies play an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in SSc patients, helping to predict the clinical outcome and optimizing patient management. Kidney transplantation may be the only treatment option available for a subset of SRC patients who develop end-stage renal failure despite aggressive angiotensin-converting enzyme inhibitor therapy. However, the posttransplant outcome for SSc patients is currently suboptimal compared to the general renal transplant population.