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Sarcoidosis primary biliary cirrhosis

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...
2016: BMC Medical Imaging
Ke Liu, Biji T Kurien, Sarah L Zimmerman, Kenneth M Kaufman, Diana H Taft, Leah C Kottyan, Sara Lazaro, Carrie A Weaver, John A Ice, Adam J Adler, James Chodosh, Lida Radfar, Astrid Rasmussen, Donald U Stone, David M Lewis, Shibo Li, Kristi A Koelsch, Ann Igoe, Mitali Talsania, Jay Kumar, Jacen S Maier-Moore, Valerie M Harris, Rajaram Gopalakrishnan, Roland Jonsson, James A Lessard, Xianglan Lu, Jacques-Eric Gottenberg, Juan-Manuel Anaya, Deborah S Cunninghame-Graham, Andrew J W Huang, Michael T Brennan, Pamela Hughes, Gabor G Illei, Corinne Miceli-Richard, Edward C Keystone, Vivian P Bykerk, Gideon Hirschfield, Gang Xie, Wan-Fai Ng, Gunnel Nordmark, Per Eriksson, Roald Omdal, Nelson L Rhodus, Maureen Rischmueller, Michael Rohrer, Barbara M Segal, Timothy J Vyse, Marie Wahren-Herlenius, Torsten Witte, Bernardo Pons-Estel, Marta E Alarcón-Riquelme, Joel M Guthridge, Judith A James, Christopher J Lessard, Jennifer A Kelly, Susan D Thompson, Patrick M Gaffney, Courtney G Montgomery, Jeffrey C Edberg, Robert P Kimberly, Graciela S Alarcón, Carl L Langefeld, Gary S Gilkeson, Diane L Kamen, Betty P Tsao, W Joseph McCune, Jane E Salmon, Joan T Merrill, Michael H Weisman, Daniel J Wallace, Tammy O Utset, Erwin P Bottinger, Christopher I Amos, Katherine A Siminovitch, Xavier Mariette, Kathy L Sivils, John B Harley, R Hal Scofield
OBJECTIVE: More than 80% of autoimmune disease predominantly affects females, but the mechanism for this female bias is poorly understood. We suspected that an X chromosome dose effect accounts for this, and we undertook this study to test our hypothesis that trisomy X (47,XXX; occurring in ∼1 in 1,000 live female births) would be increased in patients with female-predominant diseases (systemic lupus erythematosus [SLE], primary Sjögren's syndrome [SS], primary biliary cirrhosis, and rheumatoid arthritis [RA]) compared to patients with diseases without female predominance (sarcoidosis) and compared to controls...
May 2016: Arthritis & Rheumatology
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
Giulio Rossi, Alberto Cavazza, Thomas V Colby
Pathologists are frequently involved in the diagnosis of sarcoidosis on conventional biopsies or examining bronchoalveolar lavage fluid and assisting bronchoscopists when performing bronchial or transbronchial biopsies or transbronchial needle aspiration (TBNA)/endobronchial ultrasound (EBUS)-guided biopsies of enlarged lymph nodes. Histology generally does not pose difficult tasks in the correct clinical and imaging scenario, but atypical forms of sarcoidosis exist, and in these cases, the diagnosis may become difficult...
August 2015: Clinical Reviews in Allergy & Immunology
Nourdin Aqodad, Bouchra Loukili, Salim Gallouj, Taoufik Harmouch, Afaf Amarti, F Z Mernissi, Adil Ibrahimi
No abstract text is available yet for this article.
2014: Pan African Medical Journal
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
M Fallah, X Liu, J Ji, A Försti, K Sundquist, K Hemminki
BACKGROUND: The cumulative risk of non-Hodgkin lymphoma (NHL) in Sweden by age 80 years has increased to 1.1 in women and 1.6% in men in 2011. Increased risk of NHL associated with personal histories of some autoimmune diseases (ADs) is known. It is unclear whether there are other NHL-related ADs and whether this association holds across different sex, age and year of diagnosis, or NHL histological subtypes. PATIENTS AND METHODS: Over an average of 9.4-year (maximum 47 years) follow-up of 878 161 patients diagnosed in 1964-2010 with 33 different ADs, 3096 subsequent NHL were diagnosed (data: Swedish Cancer Registry)...
October 2014: Annals of Oncology: Official Journal of the European Society for Medical Oncology
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
Marina Pehlić, Dina Vrkić, Veselin Skrabić, Ana Jerončić, Gordana Stipančić, Anita Špehar Urojić, Igor Marjanac, Jasminka Jakšić, Zrinka Kačić, Vesna Boraska, Tatijana Zemunik
BACKGROUND: Common complex diseases are influenced by both genetic and environmental factors. Many genetic factors overlap between various autoimmune diseases. The aim of the present study is to determine whether four genetic variants known to be risk variants for several autoimmune diseases could be associated with an increased susceptibility to type 1 diabetes mellitus. METHODS AND FINDINGS: We genotyped four genetic variants (rs2358817, rs1049550, rs6679356, rs9865818) within VTCN1, ANXA11, IL12RB2 and LPP genes respectively, in 265 T1DM family trios in Croatian population...
2012: PloS One
J Alijotas-Reig, V Garcia-Gimenez, E Llurba, M Vilardell-Tarrés
BACKGROUND: Systemic autoimmune/granulomatous adverse reactions related to biomaterials other than silicone have rarely been reported. AIM: The aim of this paper is to communicate the cases of autoimmune/inflammatory syndrome induced by adjuvants (ASIA) in a study of Spanish patients suffering from inflammatory disorders related to biomaterial injections other than silicone, principally hyaluronic acid, acrylamides or methacrylate compounds. METHODS: The authors performed a retrospective analysis of the clinical, laboratory, histopathology and follow-up of a cohort of 250 cases of patients suffering from inflammatory/autoimmune disorders related to bioimplant injections...
October 2012: Lupus
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
Renata I Mazzucchelli, Agostino Riva, Scott K Durum
Most T cell subsets depend on IL-7 for survival. IL-7 binds to IL-7Rα and γc, initiating the signaling cascade. Deletion of IL-7Ra in humans has, for some time, been known to cause severe combined immunodeficiency. More recently, polymorphisms in IL-7R have been shown be a risk factor for a number of diseases that are autoimmune or involve excess immune and inflammatory responses including multiple sclerosis, type 1 diabetes, rheumatoid arthritis, primary biliary cirrhosis, inflammatory bowel disease, atopic dermatitis, inhalation allergy, sarcoidosis and graft-versus host disease...
June 2012: Seminars in Immunology
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
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