A case of sarcoidosis presenting with an asymptomatic elevated serum alkaline phosphatase level

SESSION TITLE: Diffuse Lung Disease Case Report Posters 9 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 02:10 pm - 02:55 pm INTRODUCTION: The diagnosis of sarcoidosis is often challenging due to multisystem involvement (1). Although lungs and lymph nodes are typically involved, hepatic sarcoidosis is also present in many patients. We present a case of sarcoidosis in an asymptomatic patient presenting with isolated elevated serum alkaline phosphatase (ALP) levels. CASE PRESENTATION: A 30-year-old Caucasian man was found to have a serum ALP level of 164 units/L (normal range 50-136) at a routine primary care visit. The rest of the liver enzyme levels were normal. He denied fevers, night sweats, nausea, vomiting, right upper quadrant pain, pruritus, or weight loss. He also denied starting any new medications. Three months later, serum ALP levels remained persistently elevated. CT imaging showed multiple pulmonary nodules, extensive axillary, abdominal, and pelvic lymphadenopathy, and splenomegaly. The liver was grossly normal in size and echogenicity. PET scan revealed numerous hypermetabolic lymph nodes and increased FDG uptake in the spleen. A normal uptake pattern was noted in the liver (figure 1). Axillary lymph node biopsy revealed noncaseating granulomatous inflammation consistent with sarcoidosis. Subsequently, he developed dyspnea on exertion from pulmonary sarcoidosis and corticosteroids were initiated. After 6 months of corticosteroid therapy, his symptoms and serum ALP levels returned to normal limits. DISCUSSION: The clinical spectrum of hepatic sarcoidosis is broad, ranging from asymptomatic disease to fulminant liver failure. About 50-65% of patients have granulomas on liver histology, however, symptomatic disease only occurs in 5-15% of patients (2). A cholestatic pattern of serum LFTs with elevated ALP levels and normal or minimally elevated transaminase levels is typically seen with hepatic sarcoidosis (2). These serum abnormalities can also be seen in asymptomatic individuals; however, it is not an indication to initiate treatment unless significant liver dysfunction or symptoms such as right upper quadrant pain, nausea, vomiting and pruritis are present. CT imaging typically shows hepatomegaly with homogenous appearing parenchyma or nodular lesions (3). Our patient presented with asymptomatic raised serum ALP levels and uncharacteristic imaging findings making the diagnosis of hepatic sarcoidosis challenging. Finally, lymph node biopsy confirmed the diagnosis. Treatment was initiated after he developed pulmonary symptoms. Although optimal therapy for hepatic sarcoidosis remains ill-defined, use of steroids has been associated with resolution of LFT abnormalities in patients. Alternatively, ursodeoxycholic acid is also used to improve cholestatic symptoms as an effective corticosteroid-sparing agent.Interestingly, patient's imaging demonstrated splenomegaly with increased uptake on PET scan in the absence of any liver abnormality. Sarcoidosis involvement of the liver and spleen often occur concurrently, and therefore splenomegaly is a clue that sarcoidosis liver involvement is present. CONCLUSIONS: Hepatobiliary involvement in sarcoidosis is very common, although it is usually asymptomatic. Isolated elevation of ALP levels can indicate the presence of hepatic sarcoidosis and may be the first clue to the diagnosis. REFERENCE #1: Spagnolo P, Luppi F, Roversi P, Cerri S, Fabbri LM, Richeldi L. Sarcoidosis: challenging diagnostic aspects of an old disease. Am J Med. 2012;125(2):118-25. REFERENCE #2: Judson MA. Hepatic, Splenic, and Gastrointestinal Involvement with Sarcoidosis. 2002; Seminars in Respiratory and Critical Care Medicine, Volume 23, Number 6. REFERENCE #3: Warshauer DM., Lee JKT. Imaging Manifestations of Abdominal Sarcoidosis. American Journal of Radiology. 2004;182. DISCLOSURES: No relevant relationships by Isam Albaba No relevant relationships by Marium Faiz No relevant relationships by Issam Halasa Consultant Removed 03/31/2023 by Marc Judson, source=Web Response Consultant relationship with Abbvie Please note: current by Marc Judson, value=Consulting fee Removed 03/31/2023 by Marc Judson, source=Web Response Consultant relationship with Xentria Please note: current by Marc Judson, value=Consulting fee Removed 03/31/2023 by Marc Judson, source=Web Response Consultant relationship with Novartis Please note: current by Marc Judson, value=Consulting fee Removed 03/31/2023 by Marc Judson, source=Web Response institutional grant support relationship with Mallinckrodt Please note: 2 years and ongoing by Marc Judson, value=institutional grant support received institution grant support relationship with Atyr Please note: 2022 Added 10/19/2022 by Marc Judson, source=Web Response, value=Grant/Research Support No relevant relationships by Fatima Mahmood