Unlabelled: Clinical presentation and histopathology of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) overlap syndrome (OS) are similar, but their management is different. We conducted a pediatric retrospective cross-sectional study of 34 patients with AIH and PSC. AIH had female predominance (74%) and was lower in PSC (45%). There was a trend toward higher frequency of blacks in PSC/OS (55%) compared to Caucasians (36%) and Hispanics (9%), but not race differences in AIH. Inflammatory bowel disease (IBD) was present in 75% of PSC/OS. Plasma cells were not specific for AIH (found in 42% of PSC). Concentric fibrosis was not reliable for PSC as was found in 46% of AIH.
Conclusion: A combination of clinical history, laboratory tests, imaging studies and liver biopsy are required to confirm and properly treat AIH and PSC. Liver biopsy should be used to grade severity and disease progression, but cannot be used alone to diagnose these conditions.
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http://dx.doi.org/10.3109/15513815.2014.898721 | DOI Listing |
Cureus
November 2024
Critical Care Medicine, Christian Medical College, Vellore, Vellore, IND.
Sjögren's syndrome (SS) is a chronic autoimmune disorder primarily affecting exocrine glands, leading to symptoms such as dry mouth and dry eyes. While SS can occur as a primary condition, it may overlap with other autoimmune diseases, complicating management. Autoimmune hepatitis (AIH), a liver disorder characterized by elevated serum globulins and liver-specific autoantibodies, can co-occur with SS, although this overlap is rare.
View Article and Find Full Text PDFThromb Haemost
December 2024
McMaster University, Hamilton, Canada.
The McMaster Immune Thrombocytopenia (ITP) Summit was an educational seminar from leading experts in immune thrombocytopenia and related disorders geared towards hematologists, internists, immunologists, and clinical and translational scientists. The focus of the Summit was to review the mechanisms, diagnosis and treatment of primary versus secondary ITP. Specific objectives were to describe the unique features of secondary ITP, and to review its mechanisms in the context of autoimmune disease and infection.
View Article and Find Full Text PDFAm J Gastroenterol
December 2024
Department of Gastroenterology, Liver Center, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Introduction: Nationwide, population-based data on the risk of extrahepatic malignancy in patients with autoimmune hepatitis (AIH) in Asian countries are scarce. This study aimed to examine the risk of developing extrahepatic malignancies in a nationwide cohort of patients with AIH.
Methods: Using claims data from the Korean National Health Insurance Service database, patients diagnosed with AIH between 2007 and 2019 (n=7,382) were matched in a 1:8 ratio with an age- and sex-matched control population (n=58,320).
Am J Gastroenterol
December 2024
University of Texas Medical Branch, Galveston Texas, USA.
Background And Aim: Data are limited regarding etiology-specific trends for delisting and re-compensation for liver disease improvement among liver transplant (LT) listed candidates in the US.
Methods And Results: A retrospective cohort (2002-2022) using UNOS database examined etiology-specific trends for delisting and re-compensation due to liver disease improvement among candidates listed for LT. Of 120,451 listings in adults, 34,444 (2002-08), 38,296 (2009-2015), 47,711 (2016-2022) were analyzed.
Cureus
December 2024
Department of Cardiology, Japanese Red Cross Maebashi Hospital, Maebashi, JPN.
When encountering severe hypoxemia that does not respond to oxygen supplementation, it is essential to consider underlying right-to-left shunting. Among various diagnostic approaches, the microbubble test via transthoracic echocardiography (TTE) is a simple, noninvasive method for detecting pulmonary arteriovenous shunts, particularly in hepatopulmonary syndrome (HPS). Although microbubbles are usually administered peripherally, using a Swan-Ganz (SG) catheter to inject microbubbles directly into the pulmonary artery may provide even more definitive diagnostic information.
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