S5930 Decompensated Cirrhosis in a Young Woman: A Diagnostic Challenge Revealing Hepatic Sarcoidosis

Introduction: Hepatic cirrhosis is an underrecognized manifestation of systemic granulomatous disease that, while most commonly affecting the lungs, also involves the liver in up to 30% of cases. Despite a high prevalence of extrapulmonary involvement, hepatic involvement is often mild or asymptomatic. In many cases, it is only identified incidentally or after the onset of liver dysfunction. Diagnosis is further hindered as no single study is definitive. Given the potential of serious liver disease, maintaining a high index of suspicion in patients with liver dysfunction is imperative. We present a 44-year-old woman evaluated for abdominal bloating, ultimately diagnosed with hepatic sarcoidosis. Case Description/Methods: A 44-year-old woman with celiac disease and prior cholecystectomy presented with progressive abdominal pain, cyclic vomiting, jaundice, facial and lower extremity edema over several days associated with unintentional weight loss. She denied alcohol use, recent travel, or a family history of liver disease. Exam showed scleral icterus, abdominal distension with diffuse tenderness, and facial swelling. The remainder of the exam was unremarkable. Labs showed macrocytic anemia (MCV 112.5), leukocytosis (WBC 19.4), elevated total bilirubin (5.2 mg/dL), alkaline phosphatase (234 U/L), aspartate aminotransferase (233 U/L), and CRP (37.7). Computed tomography and MRCP revealed hepatomegaly and hepatic steatosis without biliary obstruction. Calculated MELD and SAAG of 16 and 2, respectively, were consistent with decompensated cirrhosis and portal hypertension. Liver biopsy showed granulomatous hepatitis with severe macrovesicular steatosis and portal-portal bridging fibrosis, concerning for sarcoidosis-related cirrhosis. Other granulomatous, autoimmune, or metabolic liver diseases remained in the differential. She was managed supportively with diuretics, lactulose and a sodium-restricted diet. She was discharged in stable condition with hepatology follow-up. Discussion: Diagnosis and surveillance of hepatic sarcoidosis remains poorly standardized, and its management is often challenging. In rare cases, hepatic involvement may be the sole manifestation. The disease frequently affects periportal regions, and isolated granulomatous hepatitis may be seen. Diagnosis requires correlation of histopathology with clinical context and supporting lab data. This case underscores the importance of considering granulomatous disorders in the differential of unexplained liver dysfunction, even without pulmonary involvement.

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Publication Details

Journal
The American Journal of Gastroenterology
Published
2025-10-01
DOI
https://doi.org/10.14309/01.ajg.0001151180.75689.ee
Primary Topic
Sarcoidosis and Beryllium Toxicity Research
Type
article
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article

S5930 Decompensated Cirrhosis in a Young Woman: A Diagnostic Challenge Revealing Hepatic Sarcoidosis

Pranav Chalasani, Tripti Nagar, Pradeep Pentapurthy, Thanaa Al-hamad
The American Journal of Gastroenterology
Sarcoidosis and Beryllium Toxicity Research
article

S5930 Decompensated Cirrhosis in a Young Woman: A Diagnostic Challenge Revealing Hepatic Sarcoidosis

Pranav Chalasani, Tripti Nagar, Pradeep Pentapurthy, Thanaa Al-hamad
article en

Abstract

Introduction: Hepatic cirrhosis is an underrecognized manifestation of systemic granulomatous disease that, while most commonly affecting the lungs, also involves the liver in up to 30% of cases. Despite a high prevalence of extrapulmonary involvement, hepatic involvement is often mild or asymptomatic. In many cases, it is only identified incidentally or after the onset of liver dysfunction. Diagnosis is further hindered as no single study is definitive. Given the potential of serious liver disease, maintaining a high index of suspicion in patients with liver dysfunction is imperative. We present a 44-year-old woman evaluated for abdominal bloating, ultimately diagnosed with hepatic sarcoidosis. Case Description/Methods: A 44-year-old woman with celiac disease and prior cholecystectomy presented with progressive abdominal pain, cyclic vomiting, jaundice, facial and lower extremity edema over several days associated with unintentional weight loss. She denied alcohol use, recent travel, or a family history of liver disease. Exam showed scleral icterus, abdominal distension with diffuse tenderness, and facial swelling. The remainder of the exam was unremarkable. Labs showed macrocytic anemia (MCV 112.5), leukocytosis (WBC 19.4), elevated total bilirubin (5.2 mg/dL), alkaline phosphatase (234 U/L), aspartate aminotransferase (233 U/L), and CRP (37.7). Computed tomography and MRCP revealed hepatomegaly and hepatic steatosis without biliary obstruction. Calculated MELD and SAAG of 16 and 2, respectively, were consistent with decompensated cirrhosis and portal hypertension. Liver biopsy showed granulomatous hepatitis with severe macrovesicular steatosis and portal-portal bridging fibrosis, concerning for sarcoidosis-related cirrhosis. Other granulomatous, autoimmune, or metabolic liver diseases remained in the differential. She was managed supportively with diuretics, lactulose and a sodium-restricted diet. She was discharged in stable condition with hepatology follow-up. Discussion: Diagnosis and surveillance of hepatic sarcoidosis remains poorly standardized, and its management is often challenging. In rare cases, hepatic involvement may be the sole manifestation. The disease frequently affects periportal regions, and isolated granulomatous hepatitis may be seen. Diagnosis requires correlation of histopathology with clinical context and supporting lab data. This case underscores the importance of considering granulomatous disorders in the differential of unexplained liver dysfunction, even without pulmonary involvement.

The American Journal of GastroenterologyVol. 120(10S2)
Openalex Percentile: Top 51%
Sarcoidosis and Beryllium Toxicity Research
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