Hepatic sarcoidosis with symptomatic portal hypertension: A report of 12 cases with review of the literature.

Autor:	Fauter M; Department of Internal Medicine, Croix-Rousse Hospital, Lyon, France., Rossi G; Department of Internal Medicine, Henri-Mondor Hospital, Assistance Publique - Hôpitaux de Paris, Université Paris-Est Créteil, Créteil, France., Drissi-Bakhkhat A; Department of Internal Medicine, Croix-Rousse Hospital, Lyon, France., Latournerie M; Department of Hepato-Gastroenterology, Centre Hospitalier Universitaire Dijon Bourgogne, Dijon, France., Gerfaud-Valentin M; Department of Internal Medicine, Croix-Rousse Hospital, Lyon, France., Durieu I; Department of Internal Medicine, Centre Hospitalier Universitaire Lyon Sud, Pierre-Bénite, France.; RESHAPE, INSERM U1290, Lyon University, University Claude-Bernard Lyon 1, Lyon, France., Jamilloux Y; Department of Internal Medicine, Croix-Rousse Hospital, Lyon, France.; INSERM U1111, Centre International de Recherche en Infectiologie/International Research Center in Infectiology (CIRI), University Claude-Bernard Lyon 1, Villeurbanne, France., Bailly F; Department of Hepato-Gastroenterology, Croix-Rousse Hospital, Lyon, France., Mahevas M; Department of Internal Medicine, Henri-Mondor Hospital, Assistance Publique - Hôpitaux de Paris, Université Paris-Est Créteil, Créteil, France., Sève P; Department of Internal Medicine, Croix-Rousse Hospital, Lyon, France.; RESHAPE, INSERM U1290, Lyon University, University Claude-Bernard Lyon 1, Lyon, France.; Pôle IMER, Hospices Civils de Lyon, Lyon, France.
Jazyk:	angličtina
Zdroj:	Frontiers in medicine [Front Med (Lausanne)] 2022 Dec 22; Vol. 9, pp. 995042. Date of Electronic Publication: 2022 Dec 22 (Print Publication: 2022).
DOI:	10.3389/fmed.2022.995042
Abstrakt:	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. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2022 Fauter, Rossi, Drissi-Bakhkhat, Latournerie, Gerfaud-Valentin, Durieu, Jamilloux, Bailly, Mahevas and Sève.)
Databáze:	MEDLINE
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