Clinical Reviews in Allergy & Immunology

, Volume 40, Issue 2, pp 84–91

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

  • Luc Mouthon
  • Alice Bérezné
  • Guillaume Bussone
  • Laure-Hélène Noël
  • Peter M. Villiger
  • Loïc Guillevin
Article

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.

Keywords

Scleroderma renal crisis Systemic sclerosis Hypertension Acute renal failure Angiotensin-converting enzyme inhibitors 

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Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • Luc Mouthon
    • 1
    • 4
  • Alice Bérezné
    • 1
  • Guillaume Bussone
    • 1
  • Laure-Hélène Noël
    • 2
  • Peter M. Villiger
    • 3
  • Loïc Guillevin
    • 1
  1. 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 DescartesParisFrance
  2. 2.Necker hospital, INSERM U507Paris-Descartes UniversityParisFrance
  3. 3.Department of Rheumatology, Clinical Immunology and AllergologyUniversity Hospital and University of BernBernSwitzerland
  4. 4.Department of Internal MedicineCochin HospitalParis Cedex 14France

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