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

Mouthon, Luc; Bérezné, Alice; Bussone, Guillaume; Noël, Laure-Hélène; Villiger, Peter M; Guillevin, Loïc (2009). Scleroderma Renal Crisis: A Rare but Severe Complication of Systemic Sclerosis. Clinical reviews in allergy & immunology, 40(2), pp. 84-91. Totowa, NJ: Humana Press 10.1007/s12016-009-8191-5

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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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology and Immunology

UniBE Contributor:

Villiger, Peter Matthias

ISSN:

1080-0549

Publisher:

Humana Press

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 15:11

Last Modified:

05 Dec 2022 14:22

Publisher DOI:

10.1007/s12016-009-8191-5

PubMed ID:

20012923

Web of Science ID:

000287243900003

BORIS DOI:

10.48350/31404

URI:

https://boris.unibe.ch/id/eprint/31404 (FactScience: 195911)

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