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QJM Advance Access originally published online on June 29, 2007
QJM 2007 100(8):485-494; doi:10.1093/qjmed/hcm052
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© 2007 The Author(s)
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (<http://creativecommons.org/licenses/by-nc/2.0/uk/4>) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Scleroderma renal crisis: patient characteristics and long-term outcomes

H. Penn1, A.J. Howie2, E.J. Kingdon3, C.C. Bunn4, R.J. Stratton1, C.M. Black1, A. Burns5 and C.P. Denton1

From the 1Departments of Rheumatology, 4Clinical Immunology, and 5Nephrology, Royal Free Hospital, London, 2Department of Pathology, University College London, London, 3Department of Nephrology, Royal Sussex County Hospital, Brighton, UK

Address correspondence to Dr H. Penn, Department of Rheumatology, Royal Free & University College Medical School, Pond Street, London NW3 2QG. email: h.penn{at}medsch.ucl.ac.uk

Received 15 January 2007 and in revised form 15 March 2007


  Abstract

Background: Scleroderma renal crisis (SRC) is an important complication of systemic sclerosis, causing acute renal failure, and usually hypertension.

Aims: To review the clinical and pathological features of SRC, and correlate them with renal outcomes and mortality.

Design: Retrospective case series.

Methods: We identified 110 cases of SRC managed at a single centre between 1990 and 2005.

Results: SRC occurred in 5% of scleroderma cases under follow-up. Cases were predominantly female (81%), with diffuse cutaneous disease (78%). RNA polymerase antibodies were found in 59% of cases tested. Almost all (108/110) received treatment with ACE inhibitors (ACEIs). Dialysis was not required in 36%, was required temporarily (for up to 3 years) in 23%, was required permanently in 41%. Patients not on dialysis showed improvement in estimated glomerular filtration rate after SRC (mean change +23 ml/min over 3 years). Poor renal outcome was associated with lower blood pressure at presentation, and with higher age in those requiring dialysis. Steroid use, microangiopathic haemolytic anaemia, and antibody profile were not related to renal outcome. In the 58 renal biopsies available for clinical correlation, acute changes of mucoid intimal thickening in arteries and fibrinoid necrosis in arterioles were associated with a poorer renal outcome. Mortality was high (59% survival at 5 years), and was higher in men.

Discussion: Despite the efficacy of ACEIs in managing SRC, the poor long-term outcome warrants evaluation for additional treatments for this devastating complication of systemic sclerosis.


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