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

L Teixeira¹, L Mouthon¹, A Mahr¹, A Berezné¹, C Agard², M Mehrenberger³, L-H Noël³, P Trolliet⁴, C Frances⁵, J Cabane⁶, L Guillevin¹, for the Group Français de Recherche sur le Sclérodermie (GFRS)

¹ Internal Medicine Department and Reference Center for Vasculitis and Systemic Sclerosis, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), and UPRES EA 4058, Paris-Descartes University, Faculty of Medicine, France
² Department of Internal Medicine, Hôpital Hôtel Dieu, Nantes, France
³ INSERM U507, Paris-Descartes University, Necker Hospital, Paris
⁴ Department of Nephrology, Centre Hospitalier Lyon Sud, hospices Civils de Lyon, Lyon, France
⁵ Department of Internal Medicine, Hôpital Tenon, AP-HP, Université Paris 6, Paris, France
⁶ Department of Internal Medicine, Hôpital Saint-Antoine, AP-HP, Université Paris 6, Paris, France

Luc Mouthon, Department of Internal Medicine, Cochin Hospital, 27 rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14; luc.mouthon{at}cch.aphp.fr

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 analysed using multivariate Cox proportional hazards regression models. The risk for developing SRC associated with corticosteroid (CS) exposure during the preceding 1- or 3-month periods was analysed according to a case–crossover design.

Results: A total of 50 SSc patients aged 53.3 (14.5) (mean (SD)) years were included in the study. SRC occurred between 1979 and 2003, after a mean (SD) disease duration of 27.7 (49.1) months. A total of 43 (86%) patients had diffuse SSc, 5 (10%) had limited cutaneous SSc and 2 (4%) had SSc sine scleroderma. At the time of SRC, 10 (20%) patients were taking angiotensin converting enzyme inhibitors, and mean creatininaemia was 468 (293) µmol/l. A total of 28 (56%) patients required haemodialysis. In all, 11 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 1- or 3-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.

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