IngentaConnect Scleroderma Renal Crisis, Still a Life-Threatening Complication

Authors: TEIXEIRA, LUIS¹; MAHR, ALFRED¹; BEREZNÉ, ALICE¹; NOËL, LAURE-HÉLÈNE²; GUILLEVIN, LOÏC¹; MOUTHON, LUC¹

Source: Annals of the New York Academy of Sciences, Volume 1108, Number 1, June 2007 , pp. 249-258(10)

Abstract:

Scleroderma renal crisis (SRC) is a major complication in patients with systemic sclerosis (SSc). SRC occurs during the first 4 years of disease evolution in more than 75% of the cases, almost exclusively in patients with diffuse SSc. Other risk factors, including preceding corticosteroid therapy, have been associated with an increased occurrence of SRC. Prior to the late 1970s, SRC was a major cause of death in SSc patients. However, the prognosis has dramatically improved with the introduction of angiotensin-converting enzyme inhibitors. Thus, prompt diagnosis and early initiation of angiotensin-converting enzyme inhibitors therapy will improve the outcome. Nevertheless, additional antihypertensive treatments are often needed. Quite a large proportion of patients require dialysis, although this therapy may be stopped in approximately one-third of patients. Patients remaining on dialysis after 2 years can be proposed for a renal transplantation.

Keywords: scleroderma renal crisis; systemic sclerosis; malignant hypertension; acute renal failure; thrombotic microangiopathy

Document Type: Research article

DOI: 10.1196/annals.1422.027

Affiliations: 1: Department of Internal Medicine and Reference Center for Necrotizing Vasculitis and Systemic Sclerosis, Cochin Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), and UPRES EA 4058, Paris-Descartes University, Faculty of Medicine, Paris, France 2: INSERM U507, Paris-Descartes University, Necker Hospital, Paris, France

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