Autoimmune hepatitis Histopathologic and clinical diagnosis

BACKGROUND: Nodular regenerative hyperplasia (NRH) is a rare disease that presents pathologically as diffuse hepatic nodules without fibrous septa. It is believed to be caused by vasculopathy against a background of various systemic diseases, such as hematologic, autoimmune, and drug-induced diseases, with various symptoms. In spite of the recent imaging advances, various atypical cases of nodular lesions are observed in daily clinical practice. Cases that do not completely meet these criteria are referred to as -like or -similar lesions in clinical situations, making it difficult to understand their pathogenesis...

GOALS: We aimed to examine the correlation of pre-biopsy clinical diagnosis with hepatic histopathology. BACKGROUND: Liver biopsy provides histologic information and informs physicians about the underlying clinical disease. We hypothesized that expert physicians' pre-biopsy clinical diagnoses may obviate the need for histopathological diagnosis. STUDY METHODS: Patients undergoing liver biopsy to investigate a liver diagnosis were prospectively identified...

BACKGROUND: Primary sclerosing cholangitis (PSC) manifests within a broad ethnic and racial spectrum, reflecting different levels of access to health care. AIM: To evaluate the clinical profile, complications and survival rates of patients with PSC undergoing liver transplantation (LTx) at a Brazilian reference center. METHODS: All patients diagnosed with PSC before or after LTx were included. The medical records were reviewed for demographic and clinical variables, including outcomes and survival...

PURPOSE: The gastrointestinal system is the most commonly affected organ, followed by the lungs, in patients with primary immunodeficiency disease (PID). Hence, it is common for children with PIDs to present with gastrointestinal symptoms. We aimed to analyze the clinical and histopathological findings of patients who were initially admitted to pediatric gastroenterology/hepatology clinics and subsequently diagnosed with PIDs to identify the clinical clues for PIDs. METHODS: The demographic, laboratory, and histopathological findings, treatment modality, and outcomes of patients initially admitted to the pediatric gastroenterology/hepatology unit and subsequently diagnosed with PIDs were recorded...

BACKGROUND & AIMS: Little is known about the potential impact of statins on the progression of noncirrhotic chronic liver diseases (CLDs) to severe liver disease. METHODS: Using liver histopathology data in a nationwide Swedish cohort, we identified 3862 noncirrhotic individuals with CLD and statin exposure, defined as a statin prescription filled for 30 or more cumulative defined daily doses. Statin users were matched to 3862 (statin) nonusers with CLD through direct 1:1 matching followed by propensity score matching...

Hemophagocytic lymphohistiocytosis (HLH) is a complex disease disorder that involves dysregulated activation of the immune system resulting in cytokine storm which can lead to widespread tissue injury. HLH is associated with a mortality rate of 41%. The diagnosis of HLH requires a median of 14 days to reach likely due to a varied range of symptoms and signs the disease can present with. Liver disease and HLH can have a significant overlap. Liver injury itself is frequently noticed in patients with HLH, with more than 50% of patients having elevated aspartate transaminase, alanine transaminase, and bilirubin levels...

COVID-19 exhibits diverse and systemic clinical symptoms, much like systemic autoimmune diseases, and there are notable similarities in the immune responses seen in both conditions. There are rare reports of ulcerative colitis and autoimmune hepatitis triggered by COVID-19 infection. Reported herein is a case of a previously healthy patient who was diagnosed with chronic colitis resembling ulcerative colitis, autoimmune pancreatitis, and suspected immune-mediated hepatitis (AIH-like hepatitis) 2 months after a COVID-19 infection...

BACKGROUND AND AIMS: Autoimmune liver disease (AILD) comprises of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) with a spectrum of overlap amongst the three. We analyzed the spectrum and treatment outcomes of patients with AILD presenting to a tertiary care center in India. METHODS: A retrospective analysis of AILD patients from June 2008 to April 2021 was performed. The diagnosis was based on clinical, biochemical, imaging, serological, and histological characteristics...

All the diagnostic criteria of autoimmune hepatitis (AIH) include histopathology. However, some patients may delay getting this examination due to concerns about the risks of liver biopsy. Therefore, we aimed to develop a predictive model of AIH diagnostic that does not require a liver biopsy. We collected demographic, blood, and liver histological data of unknown liver injury patients. First, we conducted a retrospective cohort study in two independent adult cohorts. In the training cohort (n = 127), we used logistic regression to develop a nomogram according to the Akaike information criterion...

INTRODUCTION: CD4+ T cells are thought to have a central role in the pathogenesis of autoimmune hepatitis (AIH). Mucosal addressin cell adhesion molecule-1 (MAdCAM-1) directs homing of CD4+ T cells in the alimentary tract and is a therapeutic target in inflammatory bowel diseases. Here we assessed MAdCAM-1 expression in AIH and viral hepatitis and related its expression with immune infiltrate analysis and histopathological key features. METHODS: Hepatic portal areas of pretreatment biopsies (n=10) and follow-up biopsies (n=9) of patients with a confirmed diagnosis of AIH were assessed for MAdCAM-1 expression and infiltrate composition using immunohistochemistry and multispectral imaging (Vectra® Polaris™)...

BACKGROUND: Plugged percutaneous liver biopsy, though has been in use for many years, is being used more frequently in patients in whom percutaneous liver biopsy is contraindicated due to proven or probable bleeding tendencies. We report our experience with this procedure, its indications, efficacy, and complications in Indian population over 2 years. METHODS: A retrospective study of 127 consecutive patients who had undergone plug liver biopsy from April 2017 to May 2019 was done from the database maintained in our department...

INTRODUCTION: Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is an extraordinary form of peripheral T-cell lymphoma, accounting for much less than 1% of all non-Hodgkin lymphoma. It usually happens amongst women with a median age of onset of 36 years. It can arise with autoimmune disease ~20%. SPTL may also coexist with different autoimmune diseases (e.g., lupus erythematosus [LE]) despite normal workup. CASE: A 44-year-old woman presented with a small, 2×2 cm mass on her right inner thigh that was erythematous, non-movable, and tender...

Drug-induced autoimmune hepatitis (DIAIH) is a specific phenotype of drug-induced liver injury that may lead to the devastating outcome of acute liver failure requiring liver transplantation. Drugs implicated in DIAIH include antimicrobials such as nitrofurantoin and minocycline, non-steroidal anti-inflammatory drugs, statins as well as anti-tumor necrosis agents. The clinical features of drug-induced liver injury are indistinguishable from idiopathic autoimmune hepatitis (AIH) as both may have positive AIH-related autoantibodies, elevated immunoglobulin G, as well as similar histopathological findings...

Autoimmune diseases (AID) are increasingly prevalent conditions which comprise more than 100 distinct clinical entities that are responsible for a great disease burden worldwide. The early recognition of these diseases is key for preventing their complications and for tailoring proper management. In most cases, autoantibodies, regardless of their potential pathogenetic role, can be detected in the serum of patients with AID, helping clinicians in making a definitive diagnosis and allowing screening strategies for early -and sometimes pre-clinical- diagnosis...

Diagnosis of AIH is based on a combination of clinical, laboratory and histological information. It has been formalised by diagnostic scoring systems, to which liver biopsy contributes substantially. Diagnostic biopsy is thus, desirable in nearly all patients. An adequate biopsy size, provision by clinicians of adequate information to histopathologists and active discussion at regular meetings are all important for accurate histological diagnosis. Recently, the specificity of some features previously thought to suggest AIH has been questioned, and new recommendations for histological diagnosis have been proposed, although not yet validated...

Autoimmune hepatitis (AIH) is an immune-mediated chronic inflammatory liver disease, characterized by hypergammaglobulinemia, the presence of specific autoantibodies, and typical abnormalities in liver histology. Prompt diagnosis and initiation of immunosuppressive treatment are necessary for both chronic and acute onset AIH to prevent progression to end-stage liver disease or fatal liver failure. However, the diagnosis of AIH is challenging mainly because of its heterogeneous clinical, serological and pathological features...

BACKGROUND AND AIMS: Wilson disease (WD) has diverse presentations that frequently mimic other liver diseases. Distinguishing WD from non-alcoholic fatty liver disease (NAFLD) and autoimmune hepatitis (AIH), can be difficult and has critical implications for medical management. This study aimed to examine the utility of histological features of WD in children compared to those with NAFLD and AIH. METHODS: A review of liver biopsy slides was performed in children with a clinical and/or genetic diagnosis of WD, seen at the Hospital for Sick Children between 1981 and 2019 and compared to controls with NAFLD and AIH...

The Immunoglobulin G4-related disease (IgG4-RD) is a multisystem inflammatory condition. Pancreaticobiliary manifestations often present as mass lesions that mimic malignancies. The diagnosis is confirmed by histopathological examination, that shows lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, and positive immunohistochemistry for IgG4. We encountered 3 such patients in a tertiary care hospital in India. Two patients presented with obstructive jaundice and the third with pain abdomen...

BACKGROUND: Primary biliary cholangitis (PBC), autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are autoimmune liver diseases associated with distinct autoantibodies. Diagnosis is based upon clinical, serological, and histopathology findings. The role of autoantibodies in the diagnosis of these autoimmune liver diseases, with the focus on PBC and AIH, will be discussed. CONTENT: When AIH or PBC is suspected, testing for multiple autoantibodies can be requested...

BACKGROUND & AIMS: Autoimmune hepatitis (AIH) is a chronic inflammatory liver condition that predominantly affects women. However, pregnancy risks remain unclear. METHODS: A nationwide population-based cohort study (ESPRESSO) in Sweden from 1992 to 2016 including 309 singleton births in women with AIH and 1532 matched births in women from the general population was performed. AIH was diagnosed as a combination of administrative coding from medical diagnosis of AIH and liver biopsy data from Sweden's 28 pathology departments...