The scleroderma kidney: progress in risk factors, therapy, and prevention.

Abstract:

: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.

journal_name

Curr Rheumatol Rep

authors

Bussone G,Bérezné A,Pestre V,Guillevin L,Mouthon L

doi

10.1007/s11926-010-0145-7

subject

Has Abstract

pub_date

2011-02-01 00:00:00

pages

37-43

issue

1

eissn

1523-3774

issn

1534-6307

journal_volume

13

pub_type

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