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Hepatic sarcoidosis primary biliary cirrhosis

Fabiano Di Marco, Giuseppina Palumbo, Silvia Terraneo, Gianluca Imeri, Elena Lesma, Nicola Sverzellati, Angela Peron, Lorenzo Gualandri, Maria Paola Canevini, Stefano Centanni
BACKGROUND: Autoimmune hepatitis/primary biliary cirrhosis overlap syndrome, lymphangioleiomyomatosis/tuberous sclerosis complex (LAM-TSC), and sarcoidosis are three rare diseases. Here we present, to the best of our knowledge, the first description of a patient with the coexistence of these three diseases. CASE PRESENTATION: A 47-year-old woman affected by LAM-TSC and primary biliary cirrosis/autoimmune hepatitis overlap syndrome. During her follow up a high resolution chest CT scan (HRTC) confirmed the presence of both multiple cysts and micronodular opacities consistent with multifocal micronodular pneumocytes hyperlasia (MMPH), and revealed multiple hilar-mediastinal symmetrical lymphadenopathies suggestive of sarcoidosis...
July 28, 2017: BMC Pulmonary Medicine
Ryan Yudistiro, Yukiko Arisaka, Azusa Tokue, Takahito Nakajima
BACKGROUND: Sarcoidosis-lymphoma syndrome (SLS) is a rare disease in which both entities coexist. We aimed to study the role of (18)F-fluorodeoxyglucose (FDG) and L-[3-(18)F] α-methyltyrosine (FAMT) positron emission tomography (PET)/computed tomography (CT) in differentiating between these two lesions. CASE PRESENTATION: A 54-year-old female with large liver tumors was referred to our Nuclear Medicine Department for staging using FDG PET/CT. She had a history of primary biliary cirrhosis (PBC) for 15 years and developed lung and mediastinal sarcoidosis 1 year before the liver tumors were noted...
January 8, 2016: BMC Medical Imaging
C Bihari, A Rastogi, N Kumar, S Rajesh, S K Sarin
AIM: The aim of this study was to investigate the clinical and pathological features of hepatic sarcoidosis in symptomatic cases. METHODS: Twenty-two symptomatic hepatic sarcoidosis cases were included in the study. Hepatic sarcoidosis was determined by typical imaging, histopathology, and high angiotensin-converting enzyme levels. Demographic data, laboratory data, imaging findings, liver biopsies, and clinical findings were analyzed. Portal hypertension (PH) was defined by the presence of ascites and/or varices; imaging findings suggestive of PH-splenomegaly (> 12 cm on longest axis); portal vein dilation (> 13 mm); collateral vessel formation; and hepatic venous pressure gradient ≥ 6 mmHg...
July 2015: Acta Gastro-enterologica Belgica
Memduh Sahin, Güldal Yılmaz, Mehmet Arhan, Ilker Sen
BACKGROUND/AIMS: Granulomas are focal aggregates of modified macrophages that are surrounded by a rim of lymphocytes and fibroblasts. The present study aimed to evaluate the prevalence and etiology of hepatic granulomas (HGs) in the Department of Gastroenterology with a wider population. MATERIALS AND METHODS: We performed a retrospective study on 2662 liver biopsy specimens analyzed between 2005 and 2011 at Gazi University Department of Gastroenterology to determine the presence of HGs...
October 2014: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
Tamara Alempijević, Aleksandra Sokić-Milutinović, Ljubisa Toncev, Aleksandra Pavlović-Marković, Srdjan Djuranović, Nada Tomanović, Jelena Drulović
INTRODUCTION: Primary biliary cirrhosis (PBC) is an immune-mediated chronic progressive inflammatory liver disease leading to destruction of small interlobular bile ducts. Sarcoidosis is a chronic disorder of unknown etiology characterized by non-caseous granulomas. CASE REPORT: We reported a 69-year-old female patient with abdominal pain, malaise, vertigo, headaches, hands tremor and partial loss of hearing. Initial laboratory findings revealed elevated liver function tests and cholesterol with positive antimytochondrial and antinuclear antibodies...
January 2014: Vojnosanitetski Pregled. Military-medical and Pharmaceutical Review
Nourr-Eddine Farouj, Jean-François D Cadranel, Ali Mofredj, Vincent Jouannaud, Maria Lahmiri, Pierre Le Lann, Alain Cazier
Sarcoidosis is a systemic granulomatous disease which may involve many organs. In approximately 95% of patients there is liver involvement, with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis. Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry. The occurrence of jaundice in sarcoidosis is rare; extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic. Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver- related death...
June 27, 2011: World Journal of Hepatology
Cumali Efe, Staffan Wahlin, Ersan Ozaslan, Alexandra Heurgue Berlot, Tugrul Purnak, Luigi Muratori, Chiara Quarneti, Osman Yüksel, Gérard Thiéfin, Paolo Muratori
AIM: To assess the prevalence of concurrent extrahepatic autoimmune diseases in patients with autoimmune hepatitis (AIH)/primary biliary cirrhosis (PBC) overlap syndrome and applicability of the 'mosaic of autoimmunity' in these patients. METHODS: The medical data of 71 AIH/PBC overlap patients were evaluated for associated autoimmune diseases. RESULTS: In the study population, 31 (43.6%) patients had extrahepatic autoimmune diseases, including autoimmune thyroid diseases (13 patients, 18...
May 2012: European Journal of Gastroenterology & Hepatology
Marcy Coash, Faripour Forouhar, Catherine H Wu, George Y Wu
Granulomas that consist of focal accumulations of macrophages are commonly found in the liver due to stimulation of the immune system by a number of agents. Manifestations are variable depending on whether the underlying cause is a systemic disease or a primary hepatic granulomatous reaction. This article describes the common causes, presentation, histopathology, and manifestations of granulomatous diseases as well as various diagnostic and management strategies.
January 2012: Journal of the Formosan Medical Association, Taiwan Yi Zhi
Bita Geramizadeh, Reza Jahangiri, Elham Moradi
BACKGROUND: Hepatic granuloma is reported in 2 - 15% of liver biopsy specimens. It is relatively easy for the pathologist to diagnose, but sometimes arriving at a specific etiology is quite difficult. Until now, there are few published studies about the etiology of hepatic granuloma in Iran. In this study, we attempt to determine the causes of hepatic granuloma from one of the largest referral centers in this country. METHODS: In a retrospective study over 12 years, a hepatopathologist reviewed all liver biopsies with granuloma...
July 2011: Archives of Iranian Medicine
Taofic Mounajjed, Amy Oxentenko, Eugenia Shmidt, Thomas Smyrk
Descriptive reports of liver histologic features in celiac disease (CD) are sparse, and the effect of a gluten-free diet (GFD) on the course of liver injury is poorly understood. We reviewed liver biopsy specimens in 30 patients with CD and performed immunostains for IgG, IgG4, IgM, and IgA. Subsequent liver biochemical tests and compliance with the GFD were recorded. Of the patients, 19 had autoimmune-mediated liver disease (AILD; autoimmune hepatitis, 9; primary sclerosing cholangitis, 7; and primary biliary cirrhosis, 3)...
July 2011: American Journal of Clinical Pathology
Jason M Vanatta, Kian A Modanlou, Amanda G Dean, Nosratollah Nezakatgoo, Luis Campos, Satheesh Nair, James D Eason
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)...
September 2011: Liver Transplantation
Sho Ogata, Takuya Horio, Yoshiaki Sugiura, Hideyuki Shimazaki, Hiroki Saito, Satoshi Aiko, Kuniaki Nakanishi, Toshiaki Kawai
Sarcoidosis is a systemic granulomatous inflammation of unknown etiology, and seems to involve the liver parenchyma in most cases. However, sarcoidosis-associated hepatocellular carcinoma is rare. We report here a case in which a hepatocellular carcinoma occurred within the liver, which was probably involved as a result of systemic sarcoidosis. A 57-year-old Japanese man had been followed up for 2 years because of diabetic nephropathy and sarcoidosis. On admission for pneumonia, imaging studies revealed an unexpected hepatic tumor...
December 2010: Acta Medica Okayama
Stephen M Lagana, Roger K Moreira, Jay H Lefkowitch
Granulomatous liver disease constitutes a category of hepatic disorders and is at present diagnosed in approximately 4% of liver biopsies. Hepatic granulomas develop through the interactions of T lymphocytes and macrophages, with the integral involvement of T-helper (T(H)) 1 or T(H)2 pathways or both, depending on the specific granulomatous disease. Hepatic granulomas may be manifested clinically by elevated levels of serum alkaline phosphatase and g-glutamyltransferase enzymes, damage to specific structures (eg, intrahepatic bile ducts in primary biliary cirrhosis), or infrequently, progressive liver disease with portal hypertension and cirrhosis (eg, sarcoidosis)...
November 2010: Clinics in Liver Disease
G Geri, P Cacoub
Liver granulomas are histopathologically defined and associated with various liver and non-livers disorders. There are five main causes of liver granulomatosis: primary biliary cirrhosis, tuberculosis, sarcoidosis, B and C viral hepatitis, and drug related. In the other cases, not associated with an underlying systemic granulomatous disease, a systematic diagnostic approach should be used to identify less common etiologies. After a careful diagnostic work-up, a long-term follow-up of patients with undetermined liver granulomatosis is mandatory as it may be a presenting feature of liver lymphoma...
September 2011: La Revue de Médecine Interne
Priyanka Jha, Liina Poder, Zhen J Wang, Antonio C Westphalen, Benjamin M Yeh, Fergus V Coakley
OBJECTIVE: The objective of this article is to provide a practical review of the conditions other than cirrhosis that can result in diffuse surface nodularity of the liver or portal hypertension. CONCLUSION: Conditions that can mimic cirrhosis on imaging include pseudocirrhosis of treated breast cancer metastases to the liver, fulminant hepatic failure, miliary metastases, sarcoidosis, schistosomiasis, congenital hepatic fibrosis, idiopathic portal hypertension, early primary biliary cirrhosis, chronic Budd-Chiari syndrome, chronic portal vein thrombosis, and nodular regenerative hyperplasia...
April 2010: AJR. American Journal of Roentgenology
Andreas Teufel, Arndt Weinmann, George J Kahaly, Catherine Centner, Anja Piendl, Marcus Wörns, Ansgar W Lohse, Peter R Galle, Stephan Kanzler
BACKGROUND: Although the pathomechanisms of autoimmune diseases in various organs remain unresolved, an accumulation of autoimmune diseases in individual patients has been observed. An overlap of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) or primary sclerosing cirrhosis has been well documented. However, the overlap of autoimmune diseases other than PBC or PSC has not yet been investigated in a large cohort. GOAL: The goal of our analysis was to investigate the incidence of concurrent autoimmune diseases in patients with AIH...
March 2010: Journal of Clinical Gastroenterology
G Martin-Blondel, B Camara, J Selves, M-A Robic, S Thebault, D Bonnet, L Alric
PURPOSE: To assess the etiologies and outcome of liver granulomatosis. METHODS: We analyzed all consecutive liver granulomatosis diagnosed in our internal medicine department from 2000 to 2008. RESULTS: Among 471 liver biopsies, 21 disclosed evidence of liver granulomatosis (4.5%), in sixteen women (76%) and five men, with a median age of 41years. Thirteen were caucasians (62%). At the time of diagnosis, six (28.5%) had isolated abnormal liver function tests, and fifteen (71...
February 2010: La Revue de Médecine Interne
M Giovinale, C Fonnesu, A Soriano, C Cerquaglia, V Curigliano, E Verrecchia, G De Socio, G Gasbarrini, R Manna
Sarcoidosis is a granulomatous disease of unknown origin, with pulmonary findings in more than 90% of patients. Extrapulmonary involvement is common and all organs can be involved (especially lymph nodes, eyes, joints, central nervous system) but it is rare to find an isolated extrapulmonary disease (less than 10% of patients). Granulomatous inflammation of the spleen and the liver is common in patients with systemic sarcoidosis, while hepatosplenic enlargement is unusual and splenic involvement rare. We report two cases of systemic sarcoidosis, that onset with splenic and hepatosplenic disease, and one case with splenic sarcoidosis without pulmonary involvement...
March 2009: European Review for Medical and Pharmacological Sciences
N Rajoriya, C J Wotton, D G R Yeates, S P L Travis, M J Goldacre
BACKGROUND: Sarcoidosis is a multi-system disorder characterised by non-caseating granulomas. Coexistence of sarcoidosis with immune-mediated and chronic inflammatory diseases has been described in case series. However, the coexistence of two different diseases in individuals can occur by chance, even if each of the diseases is rare. AIM: To determine whether sarcoidosis necessitating hospital admission or day-case care coexists with a range of immune-mediated and chronic inflammatory diseases more commonly than expected by chance...
May 2009: Postgraduate Medical Journal
Ibrahim Koral Onal, Osman Ersoy, Musa Aydinli, Ozlem Yonem, Ozgur Harmanci, Cenk Sokmensuer, Yusuf Bayraktar
BACKGROUND: Hepatic granuloma (HG) is a well defined histopathological finding with an heterogenous clinical presentation. Diagnosis of a specific clinical entity is not possible every time. Descriptive studies may shed light on the various etiologies also common and distinctive findings among these patients. METHODS: We reviewed the results of the liver biopsies of 592 patients. Characteristics of the patients with HG were extracted from the hospital charts. Laboratory studies included biochemical tests, hepatitis C virus (HCV) antibody, Brucella agglutination tests, tuberculin skin test...
November 2008: European Journal of Internal Medicine
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