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Scleroderma renal crisis.

Luc Mouthon1, Guillaume Bussone2, Alice Berezné2

  • 1From the Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP); Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; Université Paris Descartes, Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP, Paris, France.L. Mouthon, MD, PhD; G. Bussone, MD, PhD; A. Berezné, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes; L-H. Noël, MD, Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes, and Faculté de Médecine Paris Descartes, Service d'Anatomopathologie, and Service de néphrologie, hôpital Necker, AP-HP; L. Guillevin, MD, Université Paris Descartes, Service de Médecine Interne, Centre de Référence pour les vascularites nécrosantes et la sclérodermie systémique, hôpital Cochin, AP-HP, and Institut Cochin, INSERM U1016, CNRS UMR 8104, Université Paris Descartes. luc.mouthon@cch.aphp.fr.

The Journal of Rheumatology
|May 17, 2014
PubMed
Summary
This summary is machine-generated.

Scleroderma renal crisis (SRC), a severe complication of systemic sclerosis (SSc), involves malignant hypertension and acute kidney failure. Early ACE inhibitor treatment improves outcomes, but survival remains a concern for SSc patients with full SRC.

Keywords:
ACUTE RENAL FAILUREANGIOTENSIN-CONVERTING ENZYME INHIBITORSENDOTHELIN 1HYPERTENSIONSCLERODERMA RENAL CRISISSYSTEMIC SCLEROSIS

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Area of Science:

  • Nephrology
  • Rheumatology
  • Internal Medicine

Background:

  • Scleroderma renal crisis (SRC) is a serious complication of systemic sclerosis (SSc).
  • It manifests as malignant hypertension and acute kidney failure, affecting 5% of SSc patients, particularly those with diffuse disease.
  • Corticosteroid use, nephrotoxic drugs, and volume depletion can trigger SRC.

Purpose of the Study:

  • To summarize the characteristics, diagnosis, and management of Scleroderma Renal Crisis.
  • To highlight the impact of ACE inhibitors on SRC prognosis.
  • To discuss the role of dialysis and renal transplantation in SRC management.

Main Methods:

  • Review of clinical features, diagnostic criteria, and treatment strategies for SRC.
  • Analysis of prognostic factors and patient survival rates.
  • Discussion of the role of renal biopsy in atypical cases.

Main Results:

  • SRC presents with malignant hypertension and acute renal failure, often accompanied by left ventricular insufficiency and hypertensive encephalopathy.
  • Thrombotic microangiopathy is observed in 43% of cases; anti-RNA-polymerase III antibodies are present in one-third.
  • ACE inhibitors have significantly improved SRC prognosis, though 5-year survival for SSc patients with full SRC is 65%.

Conclusions:

  • Aggressive blood pressure control with ACE inhibitors is the cornerstone of SRC treatment.
  • Dialysis is often required but may be discontinued in about half of patients with good blood pressure control.
  • Renal transplantation is an option for patients requiring long-term dialysis.