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Autoimmune hepatitis Histopathologic and clinical diagnosis

Z Li, D Xu, Z Wang, Y Wang, S Zhang, M Li, X Zeng
Systemic lupus erythematosus (SLE) is a multisystem disorder which can affect the gastrointestinal (GI) system. Although GI symptoms can manifest in 50% of patients with SLE, these have barely been reviewed due to difficulty in identifying different causes. This study aims to clarify clinical characteristics, diagnosis and treatment of the four major SLE-related GI system complications: protein-losing enteropathy (PLE), intestinal pseudo-obstruction (IPO), hepatic involvement and pancreatitis. It is a systematic review using MEDLINE and EMBASE databases and the major search terms were SLE, PLE, IPO, hepatitis and pancreatitis...
January 1, 2017: Lupus
Sarah Engelhart, Robert J Glynn, Peter H Schur
PURPOSE: Immunoglobulin G4-related disease (IgG4-RD) is a relatively newly defined disease entity that refers to a group of immune-mediated disorders that have certain histopathologic, serologic, and clinical features in common. IgG4-RD is often associated with elevated serum IgG4. The discovery of IgG4-RD highlights the scarcity of literature examining elevations in other IgG subclasses and their potential associations to disease. In this retrospective chart review study, we aim to address that gap, by exploring disease associations in patients with isolated IgG subclass elevations...
March 30, 2017: Seminars in Arthritis and Rheumatism
Ying-Qiu Huang
Primary biliary cholangitis (PBC), formerly referred to as primary biliary cirrhosis, is an infrequent progressive intrahepatic cholestatic autoimmune illness that can evolve into hepatic fibrosis, hepatic cirrhosis, hepatic failure, and, in some cases, hepatocellular carcinoma. The disease itself is characterized by T-lymphocyte-mediated chronic non-suppurative destructive cholangitis and elevated serum levels of extremely specific anti-mitochondrial autoantibodies (AMAs). In this article, we will not only review epidemiology, risk factors, natural history, predictive scores, radiologic approaches (e...
November 28, 2016: World Journal of Hepatology
Venkata S Katabathina, Erin M Flaherty, Anil K Dasyam, Christine O Menias, Nicole D Riddle, Narayan Lath, Kazuto Kozaka, Osamu Matsui, Yasuni Nakanuma, Srinivasa R Prasad
On the basis of the similarities in the histopathologic findings and the clinical-biologic behaviors of select biliary and pancreatic conditions, a new disease concept, "biliary diseases with pancreatic counterparts," has been proposed. Both nonneoplastic and neoplastic pathologic conditions of the biliary tract have their counterparts in the pancreas. Immunoglobulin G4 (IgG4)-related sclerosing cholangitis is the biliary manifestation of IgG4-related sclerosing disease, and type 1 autoimmune pancreatitis is its pancreatic counterpart...
March 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Dina G Tiniakos, John G Brain, Yvonne A Bury
The diagnosis of autoimmune hepatitis (AIH) is based on a combination of biochemical, immunological and histological features and exclusion of other causes of liver disease. Typical histological features include a chronic hepatitis pattern of injury with portal inflammation and interface activity, predominance of plasma cells in the portal infiltrate, emperipolesis, and hepatocellular rosette formation. Centrilobular injury with prominent hepatocellular necrosis and mononuclear inflammation is now recognised in the histological spectrum of AIH and may represent an early stage of the disease...
2015: Digestive Diseases
Víta Žampachová, Eva Honsová
A majority of primary diseases for which orthotopic liver transplantation is carried out may recur in the liver allograft, mostly in adults. As the indication criteria, transplantation surgery and post-transplantation care improve, the patients survival lengthens as well, leading to concurrently increasing incidence as well as an increase in the relevance of recurrent diseases, which are the most significant cause of late liver graft dysfunction. The frequency, clinical consequences and therapeutic options of different disease recurrence vary considerably...
2015: Ceskoslovenská Patologie
Pari Basharat, Lisa Christopher-Stine
The idiopathic inflammatory myopathies (IIMs) comprise a group of autoimmune disorders that target skeletal muscle. They are characterized by typical laboratory and clinical features including muscle weakness, elevated muscle enzymes, characteristic histopathology of muscle biopsies, as well as electromyography abnormalities. The IIMs are divided into polymyositis, dermatomyositis, inclusion body myositis, nonspecific myositis, and immune-mediated necrotizing myopathy (IMNM). IMNM is distinguished by the absence of primary inflammation on muscle biopsy...
December 2015: Current Rheumatology Reports
Sachin Devidas Dhole, Archana S Kher, Radha G Ghildiyal, Manjusha P Tambse
AIM: The main aim of the study is to study the clinical profile of disorders of the liver and hepatobiliary system in paediatric patients and to correlate the histopathology findings of liver biopsy in chronic liver disease. Another aim being to assess the prognosis and to know the outcome and the effects of treatment in chronic liver diseases in paediatric age group. MATERIALS AND METHODS: It was a prospective study, included the clinical profile of Chronic Liver Diseases (CLD) in children and the histopathological correlation...
July 2015: Journal of Clinical and Diagnostic Research: JCDR
Vanja Vučićević Boras, Dora Gabrić, Vlaho Brailo, Nada Čikeš, Danko Velimir Vrdoljak
No abstract text is available yet for this article.
2015: Acta Dermatovenerologica Croatica: ADC
Tracey Ying, Prue Hill, Michael Desmond, John Agar, Andrew Mallett
Fibrillary glomerulonephritis is a rare cause of glomerulonephritis characterized by non-amyloid fibrillary deposits of unknown aetiology. It is generally considered idiopathic but may be associated with secondary causes such as monoclonal gammopathy, hepatitis B and C infections, autoimmune diseases and malignancies. We report two Australian families with apparent familial fibrillary glomerulonephritis inherited in an autosomal dominant pattern, and postulate the existence of a primary familial entity. Family 1 consists of an affected father and daughter; the daughter progressed to end-stage renal failure within 18 months of diagnosis, despite immunosuppressive therapy...
July 2015: Nephrology
Ufuk Bariş Kuzu, Erkin Öztaş, Nesrin Turhan, Fatih Saygili, Nuretdin Suna, Hakan Yildiz, Mustafa Kaplan, Muhammet Yener Akpinar, Meral Akdoğan, Sabite Kaçar, Zeki Mesut Yalin Kiliç, Aydin Şeref Köksal, Bülent Ödemiş, Ertuğrul Kayaçetin
AIM: Drug-induced liver injury (DILI) is becoming a worldwide problem with its still unexplained properties. METHODS: The data of patients who were diagnosed with DILI between January 2008 and December 2013 were assessed. RESULTS: Five patients had been diagnosed with intrinsic and 82 patients with idiosyncratic DILI. The most common causative agents were antimicrobial drugs. The most common injury pattern was hepatocellular. When patients with bilirubin levels of more than 5 mg/dL were divided into two groups according to receiving steroid therapy (n = 11) or not (n = 40), there was not any significant difference according to their clinical results (P > 0...
April 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
Koichi Kobayashi, Nobuharu Ohshima, Masahiro Shimada, Masahiro Kawashima, Hirotoshi Matsui, Akira Hebisawa
A 34-year-old woman visited the hospital suffering from enanthema of the tongue, hair loss, and nonproductive cough. Corticosteroid administration slightly resolved the enanthema and hair loss, but not the nonproductive cough. She was transferred to another hospital for the resection of a retroperitoneal mass, which was histopathologically diagnosed as unicentric, hyaline vascular type Castleman's disease. She was then referred to our hospital due to progressive dyspnea and was diagnosed as having bronchiolitis obliterans based on computed tomography scan findings and a lung function test, while paraneoplastic pemphigus was clinically considered for her enanthema...
September 2014: Respirology Case Reports
J Nares-Cisneros, Y Jaramillo-Rodríguez
BACKGROUND: Autoimmune hepatitis (AIH) is a chronic inflammatory disease of the liver with nonspecific clinical manifestations that causes greater liver damage in children than in adults. AIMS: To analyze the clinical progression, biochemical profiles, histopathologic changes, and treatment response in 20 children with AIH. MATERIAL AND METHODS: A retrospective study was carried out on the variables associated with clinical progression, diagnosis, and treatment response in children seen at the the Unidad Médica de Alta Especialidad (UMAE) No...
October 2014: Revista de Gastroenterología de México
Ynto S de Boer, Carin M J van Nieuwkerk, Birgit I Witte, Chris J J Mulder, Gerd Bouma, Elisabeth Bloemena
AIMS: In this study, we aimed to evaluate the use of typical histological features of both the revised original (1999) and simplified (2008) criteria in the diagnosis of autoimmune hepatitis (AIH) in clinical practice. METHODS AND RESULTS: We performed a detailed histopathological evaluation of the pretreatment biopsies of 63 AIH patients, and used biopsies of 62 untreated chronic viral hepatitis patients [hepatitis B (n = 21) or hepatitis C (n = 41)] as a reference cohort...
February 2015: Histopathology
Fernando Bessone, Natalia Poles, Marcelo G Roma
Systemic lupus erythematosus (SLE) encompass a broad spectrum of liver diseases. We propose here to classify them as follows: (1) immunological comorbilities (overlap syndromes); (2) non-immunological comorbilities associated to SLE; and (3) a putative liver damage induced by SLE itself, referred to as "lupus hepatitis". In the first group, liver injury can be ascribed to overlapping hepatopathies triggered by autoimmune mechanisms other than SLE occurring with higher incidence in the context of lupus (e.g...
June 27, 2014: World Journal of Hepatology
Takahiro Nakazawa, Itaru Naitoh, Kazuki Hayashi, Katsuyuki Miyabe, Shuya Simizu, Takashi Joh
IgG4-related sclerosing cholangitis (IgG4-SC) is often associated with autoimmune pancreatitis. However, the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis (PSC), and the presence of segmental stenosis suggests cholangiocarcinoma (CC). IgG4-SC responds well to steroid therapy, whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention. Since IgG4-SC was first described, it has become a third distinct clinical entity of sclerosing cholangitis...
November 21, 2013: World Journal of Gastroenterology: WJG
Daniel Ferraz de Campos Mazo, Graciana Bandeira Salgado de Vasconcelos, Maria Adelaide Albergaria Pereira, Evandro Sobroza de Mello, Telesforo Bacchella, Flair Jose Carrilho, Eduardo Luiz Rachid Cançado
Liver dysfunction in patients with hyperthyroidism includes abnormalities associated with the effects of thyroid hormone excess, those secondary to drug-induced liver injury, and changes resulting from concomitant liver disease. Our goal was to describe clinical, biochemical, and histopathological patterns in patients suffering from hyperthyroidism and concomitant liver dysfunction and to propose an algorithm of procedures to facilitate diagnosis and management of such cases. This study describes seven patients with liver biochemistry abnormalities detected after diagnosis of hyperthyroidism and one with undiagnosed decompensated hyperthyroidism and acute hepatitis...
2013: Clinical and Experimental Gastroenterology
Orly Sneh Arbib, Michal Cohen, Marius Braun
A 50-year-old man was investigated for painless jaundice. The histologic pattern on the liver biopsy study met the criteria of autoimmune hepatitis. Further clinical and laboratory investigation revealed multi-organ involvement, including Mikulicz's disease of the salivary glands and pancreatic insufficiency. The diagnosis of IgG4-related disease was suggested by a finding of elevated blood levels of IgG4. IgG4-related disease is an inflammatory fibrosing condition characterized by T-cell infiltration of affected organs, presence of IgG4-positive plasma cells, and elevated levels of IgG4 in serum...
December 2012: Harefuah
Ho Eun Jung, Jae Young Jang, Soung Won Jeong, Jin Nyoung Kim, Hee Yoon Jang, Yun Ju Cho, Sung Ae Woo, Sae Hwan Lee, Sang Gyune Kim, Sang-Woo Cha, Young Seok Kim, Young Deok Cho, Hong Soo Kim, Boo Sung Kim
BACKGROUND/AIMS: Primary biliary cirrhosis (PBC) is a slowly progressing autoimmune disease of the liver that is characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. Serum total bilirubin is one of the various prognostic factors that have been proposed. A recent study found that PBC with accompanying autoimmune hepatitis (AIH) carries a negative prognosis. This study examined the clinical characteristics of PBC and analyzed the factors that affect its prognosis...
December 2012: Clinical and Molecular Hepatology
Niraj Kumari, Rachana Kathuria, Anshu Srivastav, Narendra Krishnani, Ujjal Poddar, Surender K Yachha
OBJECTIVES: Autoimmune liver disease (AILD) requires a constellation of clinical, serological, biochemical, and histological findings for diagnosis. Liver biopsy forms the cornerstone for the definite diagnosis of AILD, despite histological features not being pathognomonic. Liver biopsies of AILD and nonautoimmune chronic liver disease (NACLD) were reviewed blindly to assess the role of typical histological findings in differentiating AILD from NACLD in a pediatric population. PARTICIPANTS AND METHODS: Twenty-five liver biopsies of AILD and 34 liver biopsies of NACLD were reviewed retrospectively without knowledge of the final diagnosis...
March 2013: European Journal of Gastroenterology & Hepatology
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