Isolated Hepatic Sarcoidosis Presenting With Severe Hypercalcemia

Abstract

Introduction: Sarcoidosis is an inflammatory disorder of unknown etiology that can affect various organs. Lungs, intra thoracic lymph nodes and skin are the most commonly affected organs. The prevalence of hepatic sarcoidosis ranges between 5 to 30%. However, isolated hepatic sarcoidosis is rare. Hypercalcemia in sarcoidosis varies considerably due to the varying disease course and is reported to occur between 2 to 63% cases. We report a unique care of isolated liver sarcoidosis that presented with severe parathyroid hormone (PTH) independent hypercalcemia.Case: A 58 years old woman of Asian ethnicity with a past medical history of type 1 diabetes, hypothyroidism and chronic kidney disease presented to emergency department with headaches and altered mental status. The headaches were present since 4 weeks and was associated with polyuria and polydipsia. Non contrast CT scan of brain was negative for acute intracranial process. Physical examination revealed unremarkable vital signs and physical findings except for the altered mental status on neurological exam (orientation to self only). Initial laboratory testing revealed high corrected serum calcium 12.2 (8.0- 10.1 mg/dl), acute renal failure with high serum creatinine 2.90 (0.57–1.0 mg/dl), abnormal liver panel with elevated AST 84 (5-32U/L), ALT 85 (5–33 U/L), Alkaline Phosphatase 151 (35-104U/L). Repeat corrected serum calcium was still high at 12.4 mg/dl which prompted further evaluation to search for the etiology of hypercalcemia and testing revealed 1, 25 dihydroxy vitamin D (1, 25 vit D) mediated hypercalcemia. Labs showed low PTH 14.6 (15–65 pg/ml), normal serum protein electrophoresis, low PTH-related peptide <2.0 pmol/L, low 25 hydroxy vitamin D 20 (30–100 ng/ml) and high 1, 25 vit D 96.5 (19.9–79.3 pg/ml). Imaging evaluation revealed multiple hypodense nodules in both lobes of the liver seen on ultrasound, and CT scan of chest, abdomen and pelvis was unremarkable except for the similar liver findings. Biopsy of the liver lesion revealed non-caseating granulomas with no evidence for lymphoma and negative for acid fast bacteria and fungal organisms. Patient was treated with intravenous fluids, zoledronic acid and initiated on a course on oral prednisone that was tapered over a period of 6 months. There was a tremendous improvement in overall clinical condition. Serum calcium and 1, 25 vit D levels normalized. CT scan performed 3 months later showed complete resolution of all liver lesions.Conclusion: While most cases of hepatic sarcoidosis are asymptomatic and are incidentally found due to abnormal liver function tests or imaging done for other causes, we present a case of isolated hepatic sarcoidosis diagnosed due to symptomatic severe PTH independent hypercalcemia. Even though rare, extra-pulmonary sarcoidosis should be in differentials for 1, 25 vit D mediated hypercalcemia even if thoracic imaging are unremarkable.