Objective: This large study with a long-term follow-up aimed to evaluate the clinical presentation, laboratory findings, histological profile, treatments, and outcomes of children and adolescents with autoimmune hepatitis.
Methods: The medical records of 828 children and adolescents with autoimmune hepatitis were reviewed. A questionnaire was used to collect anonymous data on clinical presentation, biochemical and histological findings, and treatments.
Results: Of all patients, 89.6% had autoimmune hepatitis-1 and 10.4% had autoimmune hepatitis-2. The female sex was predominant in both groups. The median age at symptom onset was 111.5 (6; 210) and 53.5 (8; 165) months in the patients with autoimmune hepatitis 1 and autoimmune hepatitis-2, respectively. Acute clinical onset was observed in 56.1% and 58.8% and insidious symptoms in 43.9% and 41.2% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively. The risk of hepatic failure was 1.6-fold higher for autoimmune hepatitis-2. Fulminant hepatic failure occurred in 3.6% and 10.6% of the patients with autoimmune hepatitis-1 and autoimmune hepatitis-2, respectively; the risk was 3.1-fold higher for autoimmune hepatitis-2. The gamma globulin and immunoglobulin G levels were significantly higher in autoimmune hepatitis-1, while the immunoglobulin A and C3 levels were lower in autoimmune hepatitis-2. Cirrhosis was observed in 22.4% of the patients; biochemical remission was achieved in 76.2%. The actuarial survival rate was 93.0%. A total of 4.6% underwent liver transplantation, and 6.9% died (autoimmune hepatitis-1: 7.5%; autoimmune hepatitis-2: 2.4%).
Conclusions: In this large clinical series of Brazilian children and adolescents, autoimmune hepatitis-1 was more frequent, and patients with autoimmune hepatitis-2 exhibited higher disease remission rates with earlier response to treatment. Patients with autoimmune hepatitis-1 had a higher risk of death.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.jped.2018.04.007 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Post-COVID-19 polyautoimmunity - Fact or coincidence: A case report.
Front Med (Lausanne)
March 2023
Pathology Department, University Hospital Sharjah, Sharjah, United Arab Emirates.
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.
View Article and Find Full Text PDFAutoimmune Hepatitis Leading to Liver Cirrhosis: A Case Report.
JNMA J Nepal Med Assoc
December 2022
Tribhuvan University Teaching Hospital, Maharajgunj, Kathmandu, Nepal.
Unlabelled: Autoimmune hepatitis is a rare form of chronic liver inflammation that begins as acute hepatitis and progresses to chronic liver disease. It presents with varied clinical features from acute hepatitis to chronic liver diseases like chronic viral hepatitis and alcoholic liver disease, making it difficult to diagnose in the absence of a high index of suspicion and adequate laboratory support. Autoimmune hepatitis is divided into two categories autoimmune hepatitis-1 and autoimmune hepatitis-2 based on the antibodies involved.
View Article and Find Full Text PDFRare clinical manifestations in systemic lupus erythematosus: a review on frequency and clinical presentation.
Clin Exp Rheumatol
May 2022
Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, and Department of Clinical and Experimental Medicine, University of Pisa, Italy.
Objectives: The purpose of this study was to review the frequency and clinical presentation of the rarest clinical manifestations of systemic lupus erythematosus (SLE).
Methods: A list of 6 rare SLE manifestations were defined: gastrointestinal, liver, pulmonary, cardiac, ocular and neurological manifestations. Each topic was assigned to a pair of authors to perform a literature search and article review.
Drug-induced liver injury in Switzerland: an analysis of drug-related hepatic disorders in the WHO pharmacovigilance database VigiBase from 2010 to 2020.
Swiss Med Wkly
May 2021
Department of Clinical Pharmacology and Toxicology, University Hospital Zurich (USZ), University of Zurich (UZH), Switzerland / Health Policy Research Centre, Institute of Heath, Shiraz University of Medical Sciences, Shiraz, Iran.
Aims Of The Study: Our aim was to explore drug-induced liver injury (DILI) in Switzerland using the real-world data of the global pharmacovigilance database VigiBaseÔ, with a special focus on the new drug class of checkpoint inhibitors. This is the first study investigating drug-related hepatic disorders in Switzerland in a global pharmacovigilance database.
Methods: This was a retrospective study analysing the ICSRs (individual case safety reports) of the global pharmacovigilance database VigiBaseÔ.
Immune-related adverse reactions in the hepatobiliary system: second-generation check-point inhibitors highlight diverse histological changes.
Histopathology
February 2020
Department of Pathology, University of Washington School of Medicine, Seattle, WA, USA.
Aims: Immune check-point inhibitors are known to cause immune-mediated adverse liver injury, but our knowledge is mainly based on cases treated with ipilimumab or nivolumab.
Methods And Results: Clinicopathological features of 10 patients with hepatobiliary adverse reactions caused by second-generation drugs, pembrolizumab (n = 6) and atezolizumab (n = 4), were reviewed. Liver dysfunction developed during a median period of 3.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!