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Scleroderma Renal Crisis: A Rare but Severe Complication of Systemic Sclerosis

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Abstract

Scleroderma renal crisis (SRC) is a major complication in patients with systemic sclerosis (SSc). It is characterized by malignant hypertension and oligo/anuric acute renal failure. SRC occurs in 5% of patients with SSc, particularly in the first years of disease evolution and in the diffuse form. The occurrence of SRC is more common in patients treated with glucocorticoids, the risk increasing with increasing dose. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features. Thrombotic microangiopathy is detected in 43% of the cases. Anti-RNA-polymerase III antibodies are present in one third of patients who develop SRC. Renal biopsy is not necessary if SRC presents with classical features. However, it can help to define prognosis and guide treatment in atypical forms. The prognosis of SRC has dramatically improved with the introduction of angiotensin-converting enzyme inhibitors (ACEi). However, 5 years survival in SSc patients who develop the full picture of SRC remains low (65%). SRC is often triggered by nephrotoxic drugs and/or intravascular volume depletion. The treatment of SRC relies on aggressive control of blood pressure with ACEi, if needed in combination with other types of antihypertensive drugs. Dialysis is frequently indicated, but can be stopped in approximately half of patients, mainly in those for whom a perfect control of blood pressure is obtained. Patients who need dialysis for more than 2 years qualify for renal transplantation.

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Authors and Affiliations

  1. UPRES EA 4058, Pôle de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hospital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France

    Luc Mouthon, Alice Bérezné, Guillaume Bussone & Loïc Guillevin

  2. Necker hospital, INSERM U507, Paris-Descartes University, Paris, France

    Laure-Hélène Noël

  3. Department of Rheumatology, Clinical Immunology and Allergology, University Hospital and University of Bern, Bern, Switzerland

    Peter M. Villiger

  4. Department of Internal Medicine, Cochin Hospital, 27 rue du Faubourg Saint-Jacques, 75679, Paris Cedex 14, France

    Luc Mouthon

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Correspondence to Luc Mouthon.

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Mouthon, L., Bérezné, A., Bussone, G. et al. Scleroderma Renal Crisis: A Rare but Severe Complication of Systemic Sclerosis. Clinic Rev Allerg Immunol 40, 84–91 (2011). https://doi.org/10.1007/s12016-009-8191-5

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