Primary biliary cirrhosis (PBC) is characterized by the presence of high-titer disease specific autoantibodies directed against mitochondrial antigens (AMA) of the inner mitochondrial membrane, that are members of the 2-oxo acid complex. Among numerous other autoantibodies found in PBC the focus of ongoing studies is on the PBC-specific anti-nuclear antibodies, that are of diagnostic and clinical relevance since they can be used as a 'positive tool' in the diagnosis of AMA-negative PBC while at the same time identifying a subgroup of patients with more advanced liver disease.
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Antibodies directed against bacterial antigens in sera of Polish patients with primary biliary cholangitis.
Front Cell Infect Microbiol
January 2025
Clinic of Polish Gastroenterology Foundation, Warsaw, Poland.
Background: Primary biliary cholangitis (PBC) is a cholestatic, autoimmune liver disease with the presence of characteristic autoantibodies. The aim of the work was to determine the level of antibodies directed against bacterial antigens: (anti-anti), (anti-), (anti- ) and () in sera of PBC patients. We also performed studies on the impact of the bacterial peptides on the specific antigen-antibody binding.
View Article and Find Full Text PDFComparison of Clinical and Pathologic Features of Antimitochondrial Antibodies-negative Primary Biliary Cirrhosis and Cholestatic Type Drug-induced Liver Injury.
J Clin Gastroenterol
December 2024
Department of Infectious Diseases, The First Affiliated Hospital of Nanchang University, Nanchang, China.
Aim: To compare the respective clinical and pathologic features of antimitochondrial antibodies-negative (AMA-negative) primary biliary cirrhosis (PBC) and cholestatic type drug-induced liver injury (DILI) for clinical differential diagnosis.
Patients And Methods: Clinical data from 23 patients with AMA-negative PBC and 39 patients with cholestatic type DILI, treated at our hospital between January 2013 and January 2024, were collected and retrospectively analyzed.
Results: The cholestatic type DILI group exhibited a higher incidence of malaise and abdominal pain compared with the AMA-negative PBC group.
Successful management of immunotherapy-resistant respiratory failure in anti-mitochondrial M2 antibody-positive myositis by modified lung volume recruitment therapy: A case report.
Medicine (Baltimore)
December 2024
Department of Neurology, Showa University Fujigaoka Hospital, Yokohama, Kanagawa, Japan.
Rationale: Anti-mitochondrial antibodies (AMA) M2-positive myositis can lead to severe respiratory failure. Traditional immunotherapies sometimes fail to address respiratory failure. Herein, this CARE-compliant case report described a patient with AMA-M2-positive myositis who recovered from ventilation with tracheostomy owing to immunotherapy-resistant respiratory failure to spontaneous breathing after modified lung volume recruitment (mLVR) therapy.
View Article and Find Full Text PDFDevelopment of a standardized monoclonal antibody to the inner lipoyl domain of PDC-E2 as a potential international AMA reference.
J Transl Autoimmun
December 2024
Department of Laboratory Medicine, The Fourth Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, 210031, China.
Article Synopsis
- * A new monoclonal chimeric IgG1 antibody, called 4G6, has been developed from a murine-specific antibody for the inner lipoyl domain of PDC-E2 and can recognize all AMA epitopes used in current diagnostic tests.
- * The 4G6 antibody demonstrates strong binding affinity, making it a reliable quantitative reference for AMA testing and a promising standard for international use.
Anti-mitochondrial antibodies in systemic sclerosis target enteric neurons and are associated with GI dysmotility.
medRxiv
November 2024
Division of Gastroenterology, Dept of Medicine, Beth Israel Deaconess Medical Center, United States.
Background: Most patients with systemic sclerosis (SSc) experience gastrointestinal (GI) dysmotility. The enteric nervous system (ENS) regulates GI motility, and its dysfunction causes dysmotility. A subset of SSc patients harbor autoantibodies against the M2 mitochondrial antigen (AMA).
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