Hepatic Sarcoidosis

Dr. Welvaert described an interesting case of hepatic sarcoidosis today which presented with constitutional symptoms in addition to an elevated alkaline phosphatase and gamma-glutamyl transpeptidase (GGT). We worked through a framework for elevated alkaline phosphatase which includes determining if it is hepatic (check a GGT) and creating a differetential around intrahepatic cholestasis vs. extrahepatic cholestasis. An ultrasound helps to narrow from here. We were then left with a list of infiltrative etiologies, malignancy and drug/toxins. Liver biopsy was the key to the diagnosis.

Image courtesy of slideshare.net

Image courtesy of slideshare.net

According to UpToDate, 50-65% of patients with sarcoidosis have granulomas on liver biopsy. Symptomatic sarcoid only occurs in 5-15% of instances. Abdominal pain and pruritus are noted ~15% while fever, weight loss and jaundice less than 5%. There is elevation in serum transferases in 50-70% however, the degree is less than the alkaline phosphatase. Serum angiotension converting enzyme levels may be elevated but is normal in 25% of patients therefore, is not useful diagnostically. Diagnosis is made on biopsy with noncaseating granulomas. Remember, granulomas can also be found in cases of TB, fungal infection, brucellosis, Q fever, Hodgkin disease and drug toxicity, etc.

The patient was treated with steroids. There are small trials suggesting the use of urosodeoxycholic acid in those with cholestatic jaundice with pruritus.