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Rheumatology 2009 48(Supplement 3):iii32-iii35; doi:10.1093/rheumatology/ken483
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

This article appears in the following Rheumatology issue: Ten years of partnership: translating ideas into progress in systemic sclerosis [View the issue table of contents]

Reviews

Renal complications and scleroderma renal crisis

C. P. Denton1, G. Lapadula2, L. Mouthon3 and U. Müller-Ladner4

1Centre for Rheumatology, Royal Free Hospital, London, UK, 2Department of Internal Medicine and Public Medicine, Rheumatology Unit, University of Bari, Bari, Italy, 3Hôpital Cochin, Assistance Publique des Hôpitaux de Paris (AP-HP), Paris, France and 4Department for Internal Medicine and Rheumatology, Justus-Liebig University, Giessen, Germany.

Correspondence to: C. P. Denton, Centre for Rheumatology, Royal Free Hospital, Pond Street, Rheumatology Unit, Lower Ground Floor, London NW3 2QG, UK. E-mail: c.denton{at}medsch.ucl.ac.uk


  Abstract

Scleroderma renal crisis (SRC) occurs in 5–10% of SSc patients, who may present with an abrupt onset of hypertension, acute renal failure, headaches, fevers, malaise, hypertensive retinopathy, encephalopathy and pulmonary oedema. Patients at greatest risk of developing SRC are those with diffuse cutaneous or rapidly progressive forms of SSc, and treatment with a recently commenced high dose of corticosteroid. Laboratory tests may demonstrate hypercreatinaemia, microangiopathic haemolytic anaemia (MAHA), thrombocytopaenia and hyperreninaemia. Renal crisis is also linked to a positive ANA speckled pattern, antibodies to RNA polymerase I and II, and an absence of anti-centromere antibodies. Early, aggressive treatment with angiotensin-converting enzyme inhibitors has improved prognosis in SRC, although 40% of the patients may require dialysis, and mortality at 5 yrs is 30–40%. Median time to recovery is 1 yr, and typically occurs within 3 yrs. Prognosis is worse for males, but may not be related to corticosteroid use, presence of MAHA or severity of renal pathology. Modification of endothelin over-activity, which is implicated in the pathogenesis of SRC, may offer a future therapeutic approach.

KEY WORDS: Endothelin-1, Systemic sclerosis, Scleroderma renal crisis

Submitted 20 February 2008; revised version accepted 1 December 2008.
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