STAGE 0 HEPATIC SARCOIDOSIS WITH SUPERIMPOSED PRIMARY BILIARY CIRRHOSIS

Abstract

SESSION TITLE: Systemic Disease with Diffuse Lung Symptoms Case PostersSESSION TYPE: Case Report PostersPRESENTED ON: 10/17/2022 12:15 pm - 01:15 pmINTRODUCTION: Sarcoidosis is a multisystem disease with characteristic noncaseating granulomas in the affected organs. Pulmonary involvement is the most common site with secondary involvement within the liver seen in upwards of 70% of patients. Cases of primary hepatic sarcoidosis are rare and are usually discovered incidentally. Additionally, there seems to be an underlying association between primary hepatic sarcoidosis and primary biliary cirrhosis which may confound the clinical picture even further. Here we discuss a patient with asymptomatic hypercalcemia that was found to have stage 0 hepatic sarcoidosis with coexisting primary biliary cirrhosis.CASE PRESENTATION: A 57-year-old male presented to the hospital from his PCP for asymptomatic hypercalcemia (12.2mg/dL), normocytic anemia, and renal insufficiency. PTH, PTHrP were normal, 1,25-dihydroxyvitamin D was elevated to 88 pg/mL, and 25-hydroxyvitamin low at 12.3 ng/mL. Concern was made for sarcoidosis given the hypercalcemia and Vitamin D. A chest Xray showed no hilar lymphadenopathy and angiotensin-1-converting enzyme level was normal. A skeletal survey and bone scan were negative for lytic and blastic lesions. Serum light chains, SPEP and UPEP were normal. Bone marrow bx was done and found to be normal. The patient's calcium continued to rise along with a new rise in ALP and GGT on day 5. Due to the isolated rise in ALP and GGT concern was made for primary biliary cirrhosis and antimitochondrial antibodies were found to be elevated at 45 units. A PET was done to rule out underlying malignancy causing occult lytic lesions or paraneoplastic syndrome. PET showed FDG uptake within the L hepatic lobe, image 1. Liver biopsy was performed showing noncaseating granulomas and interlobular bile duct destruction. Bx was negative for acid-fast bacilli and fungi. Findings were consistent with stage 0 sarcoidosis of the liver with superimposed primary biliary cirrhosis(PBC). The patient was subsequently started on 40mg of Prednisone and prior to discharge Ca improved to 10.1 mg/dL.DISCUSSION: This case shows that hepatic manifestation can be the first sign of sarcoidosis. Liver sarcoidosis is usually asymptomatic with minimal liver function abnormalities. However, our patient presented with hypercalcemia, renal insufficiency, anemia, and liver injury as the first signs of stage 0 hepatic sarcoidosis. ACE and chest imaging can be falsely reassuring. Additionally PBC should be high on the differential when dealing with a cholestatic liver injury. The most common abnormal liver enzyme is ALP, present in 15% of patients with histological evidence of sarcoidosis.CONCLUSIONS: Although rare, primary biliary cirrhosis has been reported in several patients with liver sarcoidosis. High clinical suspicion is needed by the physician to diagnose rare presentations of simultaneous presentations of primary hepatic sarcoidosis and primary biliary cirrhosis as seen here.Reference #1: Tadros M, Forouhar F, Wu GY. Hepatic Sarcoidosis. J Clin Transl Hepatol. 2013;1(2):87-93. doi:10.14218/JCTH.2013.00016Reference #2: Graf C, Arncken J, Lange CM, et al. Hepatic sarcoidosis: Clinical characteristics and outcome. JHEP Rep. 2021;3(6):100360. Published 2021 Sep 3. doi:10.1016/j.jhepr.2021.100360Reference #3: D'Amato D, De Vincentis A, Malinverno F, et al. Real-world experience with obeticholic acid in patients with primary biliary cholangitis. JHEP Rep. 2021;3(2):100248. Published 2021 Jan 27. doi:10.1016/j.jhepr.2021.100248DISCLOSURES: No relevant relationships by Asher GorantlaNo relevant relationships by Bhesh KarkiNo relevant relationships by Krunal Patel SESSION TITLE: Systemic Disease with Diffuse Lung Symptoms Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: Sarcoidosis is a multisystem disease with characteristic noncaseating granulomas in the affected organs. Pulmonary involvement is the most common site with secondary involvement within the liver seen in upwards of 70% of patients. Cases of primary hepatic sarcoidosis are rare and are usually discovered incidentally. Additionally, there seems to be an underlying association between primary hepatic sarcoidosis and primary biliary cirrhosis which may confound the clinical picture even further. Here we discuss a patient with asymptomatic hypercalcemia that was found to have stage 0 hepatic sarcoidosis with coexisting primary biliary cirrhosis. CASE PRESENTATION: A 57-year-old male presented to the hospital from his PCP for asymptomatic hypercalcemia (12.2mg/dL), normocytic anemia, and renal insufficiency. PTH, PTHrP were normal, 1,25-dihydroxyvitamin D was elevated to 88 pg/mL, and 25-hydroxyvitamin low at 12.3 ng/mL. Concern was made for sarcoidosis given the hypercalcemia and Vitamin D. A chest Xray showed no hilar lymphadenopathy and angiotensin-1-converting enzyme level was normal. A skeletal survey and bone scan were negative for lytic and blastic lesions. Serum light chains, SPEP and UPEP were normal. Bone marrow bx was done and found to be normal. The patient's calcium continued to rise along with a new rise in ALP and GGT on day 5. Due to the isolated rise in ALP and GGT concern was made for primary biliary cirrhosis and antimitochondrial antibodies were found to be elevated at 45 units. A PET was done to rule out underlying malignancy causing occult lytic lesions or paraneoplastic syndrome. PET showed FDG uptake within the L hepatic lobe, image 1. Liver biopsy was performed showing noncaseating granulomas and interlobular bile duct destruction. Bx was negative for acid-fast bacilli and fungi. Findings were consistent with stage 0 sarcoidosis of the liver with superimposed primary biliary cirrhosis(PBC). The patient was subsequently started on 40mg of Prednisone and prior to discharge Ca improved to 10.1 mg/dL. DISCUSSION: This case shows that hepatic manifestation can be the first sign of sarcoidosis. Liver sarcoidosis is usually asymptomatic with minimal liver function abnormalities. However, our patient presented with hypercalcemia, renal insufficiency, anemia, and liver injury as the first signs of stage 0 hepatic sarcoidosis. ACE and chest imaging can be falsely reassuring. Additionally PBC should be high on the differential when dealing with a cholestatic liver injury. The most common abnormal liver enzyme is ALP, present in 15% of patients with histological evidence of sarcoidosis. CONCLUSIONS: Although rare, primary biliary cirrhosis has been reported in several patients with liver sarcoidosis. High clinical suspicion is needed by the physician to diagnose rare presentations of simultaneous presentations of primary hepatic sarcoidosis and primary biliary cirrhosis as seen here. Reference #1: Tadros M, Forouhar F, Wu GY. Hepatic Sarcoidosis. J Clin Transl Hepatol. 2013;1(2):87-93. doi:10.14218/JCTH.2013.00016 Reference #2: Graf C, Arncken J, Lange CM, et al. Hepatic sarcoidosis: Clinical characteristics and outcome. JHEP Rep. 2021;3(6):100360. Published 2021 Sep 3. doi:10.1016/j.jhepr.2021.100360 Reference #3: D'Amato D, De Vincentis A, Malinverno F, et al. Real-world experience with obeticholic acid in patients with primary biliary cholangitis. JHEP Rep. 2021;3(2):100248. Published 2021 Jan 27. doi:10.1016/j.jhepr.2021.100248 DISCLOSURES: No relevant relationships by Asher Gorantla No relevant relationships by Bhesh Karki No relevant relationships by Krunal Patel