Atteintes rénales du lupus

Abstract

Systemic lupus erythematosus is the most characteristic of auto-immune disorders that can lead to tissue damage in many organs, including kidney. Lupus nephritis occurs in 10 to 40% of lupus patients. Its clinical hallmark is the appearance of a proteinuria as soon as a 0.5 g/g or 0.5 g/d threshold, which calls for a renal histological evaluation in order to determine the lupus nephritis severity and the need for specific therapy. More than half of renal biopsies lead to the diagnosis of active lupus nephritis–class III or class IV A according to the ISN/RPS classification–that are the most severe in regards to renal prognosis and mortality. Their treatment aims to their clinical remission and to the prevention of relapse with minimal adverse effects for eventually the preservation of renal function, the prevention of other irreversible damage, and the reduction of risk of death. The remission is obtained through induction therapies of which the association of high dose steroids and cyclophosphamide is the most experienced. When this association must be challenged by the prevention of side-effect, in particular infertility, mycophenolate can be given instead of cyclophosphamide. Maintenance therapy, for the prevention of relapse, consists in mycophenolate or in azathioprine, mycophenolate being the most efficient however associated with a high risk of teratogenicity. Withdrawal of maintenance therapy is possible after two to three years in absence of high risk factors of relapse of lupus nephritis, however a reliable assessment of the risk of relapse is still lacking. Only pure membranous lupus nephritis (pure class V) associated with high level proteinuria requires specific therapies that usually associates steroids and an immunosuppressive drug. However, their choice hierarchy and even the use of less immunosuppressive strategies remain to be determined in terms of benefice over risk ratios. In spite of its trigger effect on lupus activity, pregnancy can be safe and successful if scheduled in the lowest risk periods with close multidisciplinary monitoring before, during and after. When necessary, renal replacement therapy does not require specific adaptation, renal transplantation is the best option when possible, as early as possible.