UNFAVORABLE OUTCOMES (END-STAGE RENAL FAILURE DEATH) IN CHILDHOOD-ONSET SYSTEMIC LUPUS-ERYTHEMATOSUS - A MULTICENTER STUDY IN PARIS AND ITSENVIRONS/

Thirty of 118 cases of childhood onset systemic lupus erythematosus co llected in the Parisian area had an unfavorable outcome. Thirteen pati ents evolved to end-stage renal failure. Seven survived with renal sub stitution therapy, and 6 other patients subsequently died Most had dif fuse proliferative glomerulonephritis, the pattern of glomerular disea se classically responsible for end-stage renal failure. Three patients had membranous nephropathy with segmental lesions, a form of glomerul onephritis whose severe prognosis should be emphasized. In another pat ient, end-stage renal failure was precipitated by thrombotic microangi opathy. Seventeen other patients died and in most, the causes of death were multiple. In 7, death could be attributed to complications secon dary to an infection and in 4 other cases to SLE exacerbation with sev ere organ involvement. Two patients died suddenly, another showed card iac failure and another had malignant hypertension. Of the remaining 2 patients, one suffered anticoagulant therapy complications after trea tment for renal artery stenosis and the second, an urothelial carcinom a. Unfavorable evolutions were high among patients coming from French departments and territories, and among North African patients. One may speculate that poor outcome is associated with ethnic characteristics or with socioeconomic factors. However, the problem of compliance wit h treatment is clearly an extremely important factor in the prognosis. Both end-stage renal failure and death were in some of our cases prec ipitated by treatment interruption, indicating an insufficient underst anding of the importance of treatment in this chronic disease.