The scleroderma kidney: progress in risk factors, therapy, and prevention.
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.
Pôle de Médecine Interne, Hôpital Cochin, 27 Rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France.
Guillaume
G
Bussone
Alice
A
Bérezné
Vincent
V
Pestre
Loïc
L
Guillevin
Luc
L
Mouthon
D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D006801:Humans; D006977:Hypertension, Renal / Q000188:drug therapy* / Q000209:etiology / Q000517:prevention & control; D007674:Kidney Diseases / Q000188:drug therapy* / Q000209:etiology / Q000517:prevention & control; D011379:Prognosis; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications*
D000806:Angiotensin-Converting Enzyme Inhibitors