Acute Kidney Injury Secondary to Necrotizing Sarcoid Granulomatosis
Table 2
Teaching points.
(1) Necrotizing sarcoid granuloma (NSG) is a rare controversial entity showing features that lie in between sarcoidosis and Wegener granulomatosis with sarcoid like granulomas and extensive necrosis.
(2) The usual clinical manifestations are cough, fever, dyspnea, weight loss, night sweats, fatigue. About 40% can be asymptomatic at presentation. Extra pulmonary manifestations are seen in 30% of the patients and involvement of liver is very rare.
(3) Histologically, it shows a triad of sarcoid granulomas, vasculitis and large areas of necrosis. On Imaging a solitary mass hyperfixating in PET Scan is often seen. Differential diagnosis includes nodular sarcoidosis, Wegener’s granulomatosis and tuberculosis.
(4) Corticosteroids are mainstay of treatment. Prednisone (1 mg/kg/day) tapered over several weeks to months is recommended. In steroid resistant cases azathioprine, methotrexate and hydroxychloroquine may be used.
(5) Due to its rarity and diagnostic difficulty, treatment is challenging for clinicians, pathologists and radiologists. Prognosis is good, but relapse is common.
