Journal
CURRENT RHEUMATOLOGY REPORTS
Volume 13, Issue 1, Pages 37-43Publisher
SPRINGER
DOI: 10.1007/s11926-010-0145-7
Keywords
Systemic sclerosis; Renal crisis; Risk factors; Prevention; Treatment
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Funding
- AMPLI (Avenir Mutualiste des Professions Liberales Independantes)
- Societe Nationale Francaise de Medecine Interne
- Fonds d'Etudes et de Recherche du Corps Medical des Hopitaux de Paris
- Direction Regionale des Affaires Sanitaires et Sociales d'Ile-de-France
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Scleroderma renal crisis (SRC) is characterized by malignant hypertension, oliguric/anuric acute renal failure, and important mortality, with a 5-year survival rate of 65%. SRC occurs in 2% to 5% of patients with systemic sclerosis (SSc), particularly those with diffuse cutaneous SSc in the first years of disease evolution. Several retrospective studies have found high-dose corticosteroid therapy to be associated with increased risk of SRC, and anti-RNA-polymerase III antibodies have been detected in one third of patients with SRC. Treatment relies on the early control of blood pressure with increasing doses of angiotensin-converting enzyme inhibitors, eventually associated with calcium channel blockers together with dialysis if necessary. After 2 years on dialysis, eligible patients should be considered for renal transplantation. The strategy for prevention of SRC lacks consensus. However, corticosteroids and/or nephrotoxic drugs should be avoided in patients with diffuse cutaneous SSc.
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