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J Rheumatol. 2014 Jun;41(6):1040-8. doi: 10.3899/jrheum.131210. Epub 2014 May 15.

Scleroderma renal crisis.

Author information

1
From 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.
2
From 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.

Abstract

Scleroderma renal crisis (SRC) is characterized by malignant hypertension and oligo-anuric acute renal failure. It occurs in 5% of patients with systemic sclerosis (SSc), particularly in patients with diffuse disease during the first years. SRC is more common in patients receiving corticosteroids, the risk increasing with increasing dose. The disease is sometimes triggered by use of nephrotoxic drugs and/or intravascular volume depletion. Left ventricular insufficiency and hypertensive encephalopathy are typical clinical features. Thrombotic microangiopathy is detected in 43% of cases, and anti-RNA-polymerase III antibodies are present in one-third of patients. Renal biopsy is not necessary if SRC presents classical features. However, biopsy may help to define the prognosis and guide treatment in atypical forms. The prognosis of SRC has greatly improved with the introduction of angiotensin-converting enzyme (ACE) inhibitors. However, the 5-year survival for SSc patients with full SRC remains low (65%). The treatment of SRC relies on aggressive blood pressure control with an ACE inhibitor, combined with other antihypertensive drugs if needed. Dialysis is frequently indicated but can be stopped in about half of patients, mainly those with good blood pressure control. Patients who need dialysis for more than 2 years qualify for renal transplantation.

KEYWORDS:

ACUTE RENAL FAILURE; ANGIOTENSIN-CONVERTING ENZYME INHIBITORS; ENDOTHELIN 1; HYPERTENSION; SCLERODERMA RENAL CRISIS; SYSTEMIC SCLEROSIS

PMID:
24833760
DOI:
10.3899/jrheum.131210
[Indexed for MEDLINE]

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