Unfavorable outcomes (end-stage renal failure/death) in childhood onset systemic lupus erythematosus. A multicenter study in Paris and its environs.

Thirty of 118 cases of childhood onset systemic lupus erythematosus collected in the Parisian area had an unfavorable outcome. Thirteen patients evolved to end-stage renal failure. Seven survived with renal substitution therapy, and 6 other patients subsequently died. Most had diffuse proliferative glomerulonephritis, the pattern of glomerular disease classically responsible for end-stage renal failure. Three patients had membranous nephropathy with segmental lesions, a form of glomerulonephritis whose severe prognosis should be emphasized. In another patient, end-stage renal failure was precipitated by thrombotic microangiopathy. Seventeen other patients died and in most, the causes of death were multiple. In 7, death could be attributed to complications secondary to an infection and in 4 other cases to SLE exacerbation with severe organ involvement. Two patients died suddenly, another showed cardiac failure and another had malignant hypertension. Of the remaining 2 patients, one suffered anticoagulant therapy complications after treatment for renal artery stenosis and the second, an urothelial carcinoma. 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 with treatment is clearly an extremely important factor in the prognosis. Both end-stage renal failure and death were in some of our cases precipitated by treatment interruption, indicating an insufficient understanding of the importance of treatment in this chronic disease.