Renal sarcoidosis: clinical, laboratory, and histologic presentation and outcome in 47 patients

We conducted the current study to investigate the clinical, laboratory, and histologic features at presentation and the outcome of renal sarcoidosis (RS). Exhaustive retrospective data were collected by the French Sarcoidosis Group. Forty-seven adult patients were assessed (30 male/17 female, M/F ratio: 1.76). Median estimated glomerular filtration rate (eGFR) was 20.5 mL/min per 1.73 m(2) (range, 4-93 mL/min per 1.73 m(2)). Moderate proteinuria was found in 31 (66%) patients (median, 0.7 g/24 h; range, 0-2.7 g/24 h), microscopic hematuria in 11 (21.7%) patients, aseptic leukocyturia in 13 (28.7%) patients. Fifteen of 47 (32%) patients had hypercalcemia (>2.75 mmol/L). Eleven of the 22 (50%) patients diagnosed between June and September had hypercalcemia compared with only 4 of the 25 (16%) cases diagnosed during the other months (p < 0.001). Thirty-seven patients presented with noncaseating granulomatous interstitial nephritis (GIN), and 10 with interstitial nephritis without granulomas. Apart from hypercalcemia, the clinical phenotype was also remarkable for the high frequency of fever at presentation. All patients initially received prednisone (median duration, 18 mo), 10 received intravenous pulse methylprednisolone. eGFR increased from 20 +/- 19 to 44 +/- 24.7 mL/min per 1.73 m(2) at 1 month (p < 0.001, n = 38), to 47 +/- 19.9 mL/min per 1.73 m(2) at 1 year (p < 0.001, n = 46), to 49.13 +/- 25 mL/min per 1.73 m(2) at last follow-up (p < 0.001, n = 47). A complete response to therapy at 1 year and at last follow-up was strongly correlated with complete response at 1 month (p < 0.01). Renal function improvement was inversely related to initial histologic fibrosis score. A complete response to therapy at 1 year was strongly correlated with hypercalcemia at presentation (p = 0.003). Relapses were purely renal (n = 3) and purely extrarenal (n = 10) or both (n = 4), often a long time after presentation, with in some cases severe cardiac or central nervous system involvement. We conclude that hypercalcemia and fever at presentation are often associated with RS; RS is most often and permanently responsive to corticosteroid treatment, but some degree of persistent renal failure is highly frequent and its degree of severity in the long run is well predicted from both histologic fibrotic renal score and response obtained at 1 month.