Background:
Sarcoidosis is a chronic disease characterized by noncaseating lesions involving any organ and tissue in the body. Hypercalcemia and acute kidney injury is a common renal presentation of sarcoidosis. Necrotizing sarcoid granulomatosis (NSG) is a granulomatous disease entity which presents with nodular masses of sarcoid like granuloma which primarily effects the lungs. It is a rare necrotizing variant of sarcoidosis. Extra pulmonary presentation of NSG is very rare.
Case presentation:
We present a 36-year-old female with hypercalcemia and acute kidney injury refractory to treatment. Whole body Flourine-18-fluorodeoxyglucose positron emission tomography computed tomography (18F-FDG PET/CT) showed increased metabolic uptake with ill-defined lesions in the liver, spleen, and pelvic lymph nodes. Biopsy of the ill-defined lesions in the liver showed necrotizing granulomatous lesions without angiitis. All the markers of tuberculosis were negative and angiotensin converting enzyme levels were elevated. Patient improved with 1 mg/kg/day oral steroid therapy and is on regular follow-up with minimal dose of steroids.
Conclusion:
Necrotizing sarcoid granulomatosis (NSG) is a rare systemic granulomatous disease. Due to its rarity and diagnostic difficulty, treatment is challenging for clinicians, pathologists and radiologists. Treatment of choice for symptomatic patients is steroid therapy. Prognosis is good with complete recovery.