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PTHRP Mediated Hypercalcemia in a Unique Case of H ...
PTHRP Mediated Hypercalcemia in a Unique Case of Hepatic Sarcoidosis
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This case highlights an unusual extrapulmonary presentation of sarcoidosis causing isolated hepatic disease and PTHrP-mediated hypercalcemia.<br /><br />A 65-year-old man with hypertension, type 2 diabetes, heart failure with recovered ejection fraction, and emphysema presented with 18 months of fatigue, 15-pound weight loss, elevated alkaline phosphatase, and mild anemia. CT chest showed multiple small bilateral pulmonary nodules and mild bronchial wall thickening. Abdominal ultrasound revealed hepatomegaly without focal lesions. He later developed progressive abdominal pain, constipation, and anorexia, and was found to have severe hypercalcemia (calcium 15.4 mg/dL) with acute kidney injury and cholestatic liver enzyme elevation.<br /><br />Initial workup included endoscopy and colonoscopy, which showed H. pylori infection and multiple tubular adenomas. Because of persistent liver abnormalities, a liver biopsy was ultimately performed and demonstrated non-necrotizing granulomatous hepatitis. Other causes of granulomatous hepatitis were excluded with negative autoimmune and infectious testing. Further laboratory evaluation showed suppressed PTH, normal 25-hydroxy vitamin D, elevated 1,25-dihydroxy vitamin D, and elevated PTHrP. These findings supported a diagnosis of hepatic sarcoidosis with atypical PTHrP-mediated hypercalcemia.<br /><br />The patient was treated acutely with IV fluids, calcitonin, and pamidronate, then started on prednisone after diagnosis. He improved clinically, with reduction in symptoms and alkaline phosphatase.<br /><br />Key teaching points:<br />- Sarcoidosis can present as isolated hepatic disease with nonspecific findings such as hepatomegaly and cholestatic liver enzymes.<br />- Untreated hepatic sarcoidosis can progress to portal hypertension, cirrhosis, and liver failure.<br />- Hypercalcemia in sarcoidosis is usually non-PTH mediated, but PTHrP-mediated cases may occur and should be considered.<br />- Unexplained hepatomegaly and systemic symptoms warrant a high index of suspicion for sarcoidosis, even when presentation is atypical.
Asset Subtitle
Zaynab Marzuk
Meta Tag
Author List
Patrick Li, Zaynab Marzuk, Zaynab Marzuk
Category
Clinical Vignettes
Concept
Sarcoidosis
Concept
Hepatic Sarcoidosis
Concept
Hypercalcemia
Concept
Hepatomegaly
Concept
Cholestatic Liver Enzymes
Distinguished
Non-Finalist
Presenter Organization
FHC at NYU Langone, NYU Grossman School of Medicine
Presenting Author
Zaynab Marzuk
Track
Adult
Keywords
sarcoidosis
hepatic sarcoidosis
granulomatous hepatitis
PTHrP-mediated hypercalcemia
hypercalcemia
hepatomegaly
cholestatic liver enzymes
non-necrotizing granulomas
prednisone
extrapulmonary sarcoidosis
Sarcoidosis
Hepatic Sarcoidosis
Hypercalcemia
Hepatomegaly
Cholestatic Liver Enzymes
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