Mortality and risk factors of scleroderma renal crisis: a French retrospective study in 50 patients

Luis Teixeira ¹, Luc Mouthon ^1*, Alfred Mahr ¹, Alice Bérezné ¹, Christian Agard ², Marion Mehrenberger ³, Laure-Hélène Noël ⁴, Pierre Trolliet ⁵, Camille Frances ⁶, Jean Cabane ⁷ and Loic Guillevin ¹

¹ Department of Internal Medicine, Cochin hospital, France
² Department of Internal Medicine, Hôpital Hôtel Dieu, Nantes, France
³ INSERM U507, Paris-Descartes University, Necker hospital, Paris, France
⁴ INSERM U507, Paris-Descartes University, Necker hospital,, France
⁵ Department of Nephrology, Centre Hospitalier Lyon Sud, hospices Civils de Lyon, France
⁶ Department of dermatology, Tenon hospital, France
⁷ Department of Internal Medicine, Saint-Antoine hospital, France

^* To whom correspondence should be addressed. E-mail: luc.mouthon{at}cch.aphp.fr.

Accepted 3 June 2007

Abstract

Objectives: To describe presentation and outcome of patients with scleroderma renal crisis (SRC).

Methods: SRC was defined as rapidly progressive oliguric renal insufficiency and/or rapidly progressive arterial hypertension occurring during the course of systemic sclerosis (SSc). Chronic dialysis-free survival was analyzed using multivariate Cox proportional hazards regression models. The risk for developing SRC associated with corticosteroid (CS) exposure during the preceding 3- or 1-month periods was analyzed according to a case-crossover design.

Results: Fifty SSc patients aged of 53.3±14.5 (mean±SD) yr were included. SRC occurred between 1979 and 2003, after a mean disease duration of 27.7±49.1 mo. Forty three (86%) patients had diffuse SSc, 5(10%) had limited cutaneous SSc and 2(4%) had SSc sine sleroderma. At the time of SRC, ten (20%) patients were on with angiotensin converting enzyme inhibitors, and mean creatininemia was 468±293 µmol/l. Twenty eight (56%) patients required hemodialysis. Eleven patients underwent a renal biopsy, all of them had specific vascular lesions of SRC. Multivariate analyses retained age >53 years and normal blood pressure as independent predictors of decreased dialysis-free survival. Exposure to CS prior to SRC was identified in 30 (60%) patients. The odds ratios for developing SRC associated with CS exposure during the preceding 3- or 1-month periods were 24.1 (95% CI: 3.0-193.8) and 17.4 (95% CI: 2.1-144.0), respectively.

Conclusion: SRC remains associated with severe morbidity and mortality. CS might increase the risk of developing SRC. Further studies are needed to confirm these results.

Keywords: corticosteroids, mortality, prognosis, renal crisis, systemic sclerosis

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