Natural history of hepatic sarcoidosis and its response to treatment (original) (raw)
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Annals of Hepatology, 2006
Sarcoidosis is a multisystem disease of unknown aetiology. Histological evidence of non-caseating granulomas represents the main finding. It affects mostly young people, targeting primary the lung and hilar lymph nodes although liver involvement is often encountered. Hepatic sarcoidosis covers a broad spectrum from asymptomatic hepatic granulomas formation and slightly deranged liver function tests to clinically evident disease with cholestasis or, in advanced cases, cirrhosis and portal hypertension. Other granulomatous diseases (mainly systemic infections like tuberculosis) should be excluded prior to treatment, as longstanding corticosteroid administration is the main stem of therapy. In advanced cases, liver transplantation represents the ultimate therapeutic option.
Hepatic Involvement in Systemic Sarcoidosis
Objective: Rare disease Background: Sarcoidosis is a systemic disease that can affect any organ, including the liver. It is manifested by the presence of non-caseating granulomas within involved organs, most commonly the pulmonary, lymphatic, and hepatic system. Unlike pulmonary or lymphatic involvement, hepatic involvement is usually asymptomatic and it is un-derdiagnosed. Here, we report a case of a patient with a history of pulmonary sarcoidosis who developed he-patic sarcoidosis. Case Report: 68-year-old female with pulmonary sarcoidosis with a 2-week history of severe abdominal pain and epigas-tric tenderness presented to our center. Abdominal magnetic resonance imaging (MRI) demonstrated mild he-patic fibrosis and cirrhosis. A thorough workup was performed including a liver biopsy which showed chronic non-necrotizing granulomas consistent with sarcoidosis. She was started on prednisone and subsequently improved. The patient was symptom-free on follow-up 1 month later. Conclusions: The majority of patients with hepatic sarcoidosis are usually asymptomatic, with only 5–30% presenting with abdominal pain, jaundice, nausea, vomiting, and hepatosplenomegaly. In rare cases, hepatic sarcoidosis can lead to cholestasis, portal hypertension, cirrhosis, or Budd-Chiari syndrome. Treatment with steroids is the mainstay of therapy; however, in severe cases, patients may require liver transplantation. This case report demonstrates that hepatic sarcoidosis is a serious condition, and if not treated, can lead to portal hypertension and cirrhosis. In patients with sarcoidosis, early detection and longitudinal follow-up is important in preventing overt liver failure. 1212 Indexed in: [PMC] [PubMed] [Emerging Sources Citation Index (ESCI)] [Web of Science by Clarivate] This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0)
Management of Hepatic Sarcoidosis
Journal of Gastrointestinal and Liver Diseases
Background and Aims: Liver involvement in sarcoidosis may occur in up to 60% of all patients. As many patients experience only minor symptoms, a high number of undiagnosed cases must be assumed. In order to successfully identify patients with hepatic sarcoidosis, a throughout characterization of these patients and their course of disease is necessary. Methods: We collected 40 patients from four German centers to evaluate current treatment standards and course of disease. All of our patients underwent liver biopsy with histologically proven granulomatous hepatitis. Results: Detailed characterization of our patients showed an overall benign course of disease. Treatment was very diverse with glucocorticoids for 1 year in 55% (22/40), 5-10 years in 18% (7/40), and permanently in 18% (7/40). Other treatments included disease-modifying anti-rheumatic drugs (DMARDs), the conventional non-biological type in 53% of all patients (of these 81% received azathioprine, 46% metotrexate, 10% hydrox...
Hepatic Sarcoidosis: Natural History and Management Implications
Frontiers in Medicine
Introduction: Hepatic granulomas are common in patients with sarcoidosis, but clinically significant liver disease is uncommon and poorly studied. We aimed to characterize the frequency and clinical course of hepatic sarcoidosis in an ethnically diverse population. Methods: This is a retrospective study including all cases of hepatic sarcoidosis in a single center. The median follow-up time was 49 months (4-121). Cases were identified based on ICD-9 and ICD-10 codes for granulomatous hepatitis, sarcoidosis, and hepatic sarcoidosis. The Chi-square and Wilcoxon-signed rank tests were used as indicated to assess for differences between groups. Results: Of 286 patients with sarcoidosis, 27 had hepatic involvement; 78% were female and 48% African American. The most common pattern of liver tests abnormalities was cholestatic. Ten patients had clinically significant hepatic involvement: cirrhosis in seven (25.9%), portal hypertension in nine (33%), and portal vein thrombosis in one (3.7%). Sex, race, and ethnicity were not associated with an increased risk of hepatic involvement or symptomatic hepatic sarcoidosis. Most patients received medical treatment, most commonly oral glucocorticoids. At the end of the follow-up period, all patients were alive but two had undergone liver transplantation due to complications of hepatic sarcoidosis. Three patients with hepatic sarcoidosis had initially been classified as AMA-negative PBC. Conclusions: Hepatic sarcoidosis was found in 9.4% of patients with sarcoidosis and was clinically significant in 37% of those. Identifying and monitoring hepatic sarcoidosis is crucial given its potential complications.
Liver Transplantation, 2005
Sarcoidosis is a systemic granulomatous disease primarily involving the lungs, lymph nodes, skin, eyes and nervous system; liver involvement is asymptomatic in most cases. However, once the patient develops clinical symptoms liver disease is usually progressive and may necessitate orthotopic liver transplantation. There are a few reports of asymptomatic recurrent sarcoidosis developing within the liver allograft. We report a case of early recurrence of sarcoidosis in the liver allograft diagnosed on biopsy in a patient who presented with severe hypercalcemia, kidney dysfunction, and increase in size of abdominal lymph nodes. The liver chemistry tests were within normal limits. The patient responded well to steroid treatment by normalizing serum calcium and creatinine levels and reducing lymph node size. To date, there has been no report in the literature of symptomatic recurrence of hepatic sarcoidosis following orthotopic liver transplantation.
Diagnostic and therapeutic challenges of hepatic sarcoidosis
BMJ case reports, 2011
The authors report three cases of hepatic sarcoidosis complicated by portal hypertension in Caucasian patients. The cases varied in their presentations from normal liver function to symptomatic cholestasis and established liver cirrhosis. In one particular case the authors discovered hepatic sarcoid after a diagnostic gastroscopy for upper gastrointestinal (GI) bleeding which showed changes of portal hypertension. In all of these cases the authors gave our patients a prolonged course of immunosuppressive treatment with over all good long-term results. The authors hope that our work will provide a modern approach into treating this disease.
Liver Transplantation, 2011
Hepatic sarcoidosis is a rare indication for liver transplantation. Using the United Network for Organ Sharing (UNOS)/Organ Procurement and Transplantation Network (OPTN) database, we evaluated patient and graft survival after orthotopic liver transplantation for sarcoidosis between October 1987 and December 2007. We assessed the potential prognostic value of multiple demographic and clinical variables, and we also compared these patients to a case-matched group of patients with primary sclerosing cholangitis (PSC) or primary biliary cirrhosis (PBC). The 1-and 5-year survival rates for the sarcoidosis group were 78% and 61%, respectively, and these rates were significantly worse than the rates for the PSC/PBC group (P ¼ 0.001). Disease recurrence in the liver is a rare cause of graft loss or patient death. Three deaths occurred in the sarcoidosis group because of recurrent hepatic sarcoidosis, and 1 death was a result of cardiac sarcoidosis. A univariate analysis identified an increasing donor risk index as a significant negative factor for outcomes for the sarcoidosis group [hazard ratio (HR) ¼ 2.06, confidence interval (CI) ¼ 1.04-4.06, P ¼ 0.037], but this finding was not found in a multivariate analysis, in which no independent predictors were found to have a significant impact. A case-matched univariate analysis demonstrated that sarcoidosis and morbid obesity were significant negative factors for outcomes, and in a multivariate analysis, sarcoidosis continued to predict worse outcomes (HR ¼ 2.39, CI ¼ 1.21-4.73, P ¼ 0.012). In conclusion, an analysis of the UNOS/OPTN database indicates that the patient and allograft survival rates for hepatic sarcoidosis are satisfactory, but they are worse in comparison with the rates for other cholestatic liver diseases.
Clinical Features and Prognostic Significance of Liver Involvement in Sarcoidosis
European respiratory & pulmonary diseases, 2016
Background: Sarcoidosis is a systemic disease, characterised by non-caseating granulomas in various organs. Liver involvement is not an uncommon feature of sarcoidosis. However, firm data establishing the clinical features and prognosis in these patients are lacking, although the incidence of liver sarcoidosis is high. In this study, we aimed to investigate the features of liver involvement in patients with sarcoidosis. Another aim was to evaluate the prognostic significance of liver disease by comparing the differences between patients with no liver involvement, limited and diffuse liver involvement. Methods: We conducted a retrospective study to evaluate the clinical and laboratory findings of 271 sarcoidosis patients seen at our institution. Patients with liver sarcoidosis identified by liver biopsy were compared to patients without liver involvement for clinical features and prognosis. The biopsy sample was considered positive if it demonstrated non-caseaiting granulomas with negative fungal and mycobacterial cultures. The patients were classified into three groups according to the radiological and histopathological biopsy results: no liver involvement; limited liver involvement: two sites positive; and diffuse liver involvement: three or more sites positive for granulomas. Results: Hepatic involvement was identified by biopsy in 81 (81/271, 29.8%) while 43 (43/271, 15.8%) patients had elevated liver function tests. Most of the patients (94.6%) had lung involvement. Other organs involved, in the order of frequency, were: lymph nodes, skin, eye, spleen and parotid gland. There was no significant difference between the three groups for forced expiratory volume in one second, forced vital capacity, total lung capacity, diffusing capacity of the lungs for carbon monoxide/alveolar volume, serum and 24-hour urinary calcium levels. Serum angiotensin-converting enzyme levels were significantly higher (p<0.01) in patients with diffuse liver involvement than patients with no or limited liver disease. Progressive disease was more frequent (p<0.01) in patients with diffuse liver disease. Extra-pulmonary organ involvement was significantly different between the three groups (p<0.01). Conclusions: Diffuse liver involvement in sarcoidosis is a significant risk factor for progressive disease. Patients with diffuse liver disease have a worse prognosis than patients without or with limited liver involvement. Diffuse hepatic involvement in sarcoidosis is also associated with an increased incidence of extra-pulmonary organ involvement. Presence of diffuse liver disease may reveal a severe prognosis for sarcoidosis patients.
Liver Transplantation, 2011
Hepatic sarcoidosis is a rare indication for liver transplantation. Using the United Network for Organ Sharing (UNOS)/Organ Procurement and Transplantation Network (OPTN) database, we evaluated patient and graft survival after orthotopic liver transplantation for sarcoidosis between October 1987 and December 2007. We assessed the potential prognostic value of multiple demographic and clinical variables, and we also compared these patients to a case-matched group of patients with primary sclerosing cholangitis (PSC) or primary biliary cirrhosis (PBC). The 1-and 5-year survival rates for the sarcoidosis group were 78% and 61%, respectively, and these rates were significantly worse than the rates for the PSC/PBC group (P ¼ 0.001). Disease recurrence in the liver is a rare cause of graft loss or patient death. Three deaths occurred in the sarcoidosis group because of recurrent hepatic sarcoidosis, and 1 death was a result of cardiac sarcoidosis. A univariate analysis identified an increasing donor risk index as a significant negative factor for outcomes for the sarcoidosis group [hazard ratio (HR) ¼ 2.06, confidence interval (CI) ¼ 1.04-4.06, P ¼ 0.037], but this finding was not found in a multivariate analysis, in which no independent predictors were found to have a significant impact. A case-matched univariate analysis demonstrated that sarcoidosis and morbid obesity were significant negative factors for outcomes, and in a multivariate analysis, sarcoidosis continued to predict worse outcomes (HR ¼ 2.39, CI ¼ 1.21-4.73, P ¼ 0.012). In conclusion, an analysis of the UNOS/OPTN database indicates that the patient and allograft survival rates for hepatic sarcoidosis are satisfactory, but they are worse in comparison with the rates for other cholestatic liver diseases.