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Ann Rheum Dis doi:10.1136/ard.2006.066985

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

  1. Luis Teixeira (luisteixeira{at}free.fr)
  1. Department of Internal Medicine, Cochin hospital, France
    1. Luc Mouthon (luc.mouthon{at}cch.aphp.fr)
    1. Department of Internal Medicine, Cochin hospital, France
      1. Alfred Mahr
      1. Department of Internal Medicine, Cochin hospital, France
        1. Alice Bérezné
        1. Department of Internal Medicine, Cochin hospital, France
          1. Christian Agard
          1. Department of Internal Medicine, Hôpital Hôtel Dieu, Nantes, France
            1. Marion Mehrenberger
            1. INSERM U507, Paris-Descartes University, Necker hospital, Paris, France
              1. Laure-Hélène Noël
              1. INSERM U507, Paris-Descartes University, Necker hospital,, France
                1. Pierre Trolliet
                1. Department of Nephrology, Centre Hospitalier Lyon Sud, hospices Civils de Lyon, France
                  1. Camille Frances
                  1. Department of dermatology, Tenon hospital, France
                    1. Jean Cabane
                    1. Department of Internal Medicine, Saint-Antoine hospital, France
                      1. Loic Guillevin
                      1. Department of Internal Medicine, Cochin hospital, France
                        • Published Online First 8 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.

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