ABSTRACT
BACKGROUND AND AIM: Granulomatous hepatitis (GH) is associated with various aetiologies, especially inflammatory and infectious disorders. Sarcoidosis is a granulomatous disease in which the liver is the fourth most affected organ. Since epithelioid cell granulomas are not specific to sarcoidosis and since most patients with hepatic sarcoidosis are asymptomatic, valuable diagnostic biomarkers are needed to support the diagnosis of sarcoidosis. This study proposes to assess the diagnostic value of serum angiotensin converting enzyme (sACE) and lymphopenia in GH for sarcoidosis. METHODS: We retrospectively analyzed the records of 90 patients referred to the internal medicine or hepatogastroenterology departments of the Lyon University Hospital (Lyon, France) between March 2002 and January 2020 in a context of GH. RESULTS: In our tertiary center, 38 patients with sarcoidosis were identified among 73 patients with GH. Lymphopenia had a high specificity (85.7%), which increased when combined with elevated (97.0%). Interestingly, specificity increased in patients under 50 years old (100%). CONCLUSIONS: Those results suggests that lymphopenia and sACE may be valuable biomarkers for sarcoidosis diagnosis in GH when combined, especially in younger patients.
ABSTRACT
Introduction: Sarcoidosis is a systemic granulomatosis of unknown etiology, characterized by the presence of immune granulomas. Liver damage is a relatively common extra-pulmonary manifestation, occurring in 3.6-30% of cases. Some patients can develop symptomatic portal hypertension (PH). Few series have evaluated the prognosis of symptomatic PH as well as the efficacy and safety of specific treatment on this complication. Methods: This is a multicenter retrospective study of cases of histologically proven hepatic sarcoidosis with symptomatic PH (ascites, digestive hemorrhage) and/or hepatic encephalopathy. Demographic characteristics, comorbidities, clinical manifestations of sarcoidosis, biological data, imaging study of the liver, treatment, and clinical outcomes were collected. Results: Twelve patients were identified, with a mean follow-up of 140 months. The M/F ratio was 1 and Caucasian origin was the most represented (75%). Seven patients presented with hepatic comorbidities: metabolic syndrome, chronic alcoholism or chronic viral hepatitis. Apart from hepatic involvement, mediastino-pulmonary involvement was the most common followed by osteoarticular and skin. Liver damage was inaugural in two thirds of cases. Nine patients developed ascites, six presented esophageal varices complicated by gastrointestinal bleeding. Three patients presented with both ascites and variceal bleeding. One case of hepatic encephalopathy was observed. Five patients presented signs of hepatocellular insufficiency during follow-up, of whom three had hepatic comorbidities. Eight out of 12 patients required second-line treatment after failure of corticosteroids, three patients underwent ligation of esophageal varices but with recurrent digestive bleeding in all cases. Two patients benefited from a transjugular intrahepatic portosystemic shunt (TIPS), also with poor result. At the end of follow-up, five patients were alive and seven patients died. Two patients received a liver transplant, with good result and without recurrence of sarcoidosis on the transplant thereafter. Two patients had quiet sarcoidosis on low dose of corticosteroids and one patient was lost to follow-up. Conclusion: Symptomatic PH related to hepatic sarcoidosis is a severe complication, with high morbidity and mortality, and frequent failure of specific treatments of PH. Early management of these patients, with detection of hepatic comorbidities seems important. In case of therapeutic failure, liver transplantation is an option to consider.
