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The Scleroderma Kidney: Progress in Risk Factors, Therapy, and Prevention

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Abstract

Scleroderma renal crisis (SRC) is characterized by malignant hypertension, oliguric/anuric acute renal failure, and important mortality, with a 5-year survival rate of 65%. SRC occurs in 2% to 5% of patients with systemic sclerosis (SSc), particularly those with diffuse cutaneous SSc in the first years of disease evolution. Several retrospective studies have found high-dose corticosteroid therapy to be associated with increased risk of SRC, and anti-RNA-polymerase III antibodies have been detected in one third of patients with SRC. Treatment relies on the early control of blood pressure with increasing doses of angiotensin-converting enzyme inhibitors, eventually associated with calcium channel blockers together with dialysis if necessary. After 2 years on dialysis, eligible patients should be considered for renal transplantation. The strategy for prevention of SRC lacks consensus. However, corticosteroids and/or nephrotoxic drugs should be avoided in patients with diffuse cutaneous SSc.

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Acknowledgments

Dr. Bussone has received financial support from AMPLI (Avenir Mutualiste des Professions Libérales & Indépendantes), the Société Nationale Française de Médecine Interne, the Fonds d’Etudes et de Recherche du Corps Médical des Hôpitaux de Paris, and the Direction Régionale des Affaires Sanitaires et Sociales d’Ile-de-France. Drs. Bérezné, Guillevin, and Mouthon are members of the Groupe Français de Recherche sur la Sclérodermie.

Disclosure

Dr. Guillevin has served as a consultant for Actelion Pharmaceuticals and GlaxoSmithKline.

Dr. Mouthon has served as a consultant for Actelion Pharmaceuticals, Pfizer, GlaxoSmithKline, and Eli Lilly and Company. Drs. Bussone, Bérezné, and Pestre reported no potential conflicts of interest relevant to this article.

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Authors and Affiliations

  1. Pôle de Médecine Interne, Hôpital Cochin, 27 Rue du Faubourg Saint-Jacques, 75679, Paris Cedex 14, France

    Guillaume Bussone, Alice Bérezné, Vincent Pestre, Loïc Guillevin & Luc Mouthon

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  1. Guillaume Bussone
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  2. Alice Bérezné
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  3. Vincent Pestre
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  4. Loïc Guillevin
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  5. Luc Mouthon
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Correspondence to Luc Mouthon.

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Bussone, G., Bérezné, A., Pestre, V. et al. The Scleroderma Kidney: Progress in Risk Factors, Therapy, and Prevention. Curr Rheumatol Rep 13, 37–43 (2011). https://doi.org/10.1007/s11926-010-0145-7

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