Renal sarcoidosis.

Renal sarcoidosis (RS) is a rare form of sarcoidosis that results in granulomatous inflammation of renal parenchyma. We describe the epidemiology, pathogenesis, clinical features, diagnostic approach, treatment strategies and outcomes of this condition. RS occurs most commonly at the time of initial presentation of sarcoidosis but can at any time along the course of the disease. The most common presenting clinical manifestations of RS are renal insufficiency or signs of general systemic inflammation. End-stage renal disease (ESRD) requiring dialysis is a rare initial presentation of RS. The diagnosis of RS should be considered in patients who present with renal failure and have either a known diagnosis of sarcoidosis or have extra-renal features consistent with sarcoidosis. A renal biopsy helps to establish the diagnosis of RS, with interstitial non-caseating granulomas confined primarily to the renal cortex being the hallmark pathological finding. However, these histologic findings are not specific for sarcoidosis, and alternative causes for granulomatous inflammation of the renal parenchyma should be excluded. Corticosteroids are the drug of choice for RS. Although RS usually responds well to corticosteroids, the disease may have a chronic course and require long-term immunosuppressive therapy. The risk of progression to ESRD is rare.
(© The Author(s) 2022. Published by Oxford University Press on behalf of the ERA.)