Renal Granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature

We analysed retrospectively 11 children with renal granulomatous sarcoidosi s confirmed by renal histology in order to describe the course and prognosi s of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10 .1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild p roteinuria, seven had transient sterile leucocyturia, four showed microscop ic haematuria, seven had a urinary concentrating defect, and enlarged kidne ys were seen in three patients. One child had hypercalcaemia and hypercalci uria, none had hypertension. Light microscopy of the kidney showed intersti tial infiltration by mononuclear cells in all children, interstitial fibros is in nine patients, epithelioid granulomas in seven, tubular involvement i n eight, and mild glomerular involvement in seven patients. Renal immunoflu orescence was negative. Ten children received prednisone for 1-11 years. Af ter a mean follow up of 5.5 years, three patients had entered end-stage ren al failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. Conclusion Renal failure, proteinuria, leucocyturia, haematuria, and concen tration defect are the prominent features of renal granulomatous sarcoidosi s in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.