ArticleFrontiers in immunology2022
Antigen Reactivity and Clinical Significance of Autoantibodies Directed Against the Pyruvate Dehydrogenase Antigen Complex in Patients With Connective Tissue Disease.
Article in Frontiers in immunology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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7 citing papers in PubMed, 10 citations in OpenAlex.
- Prevalence and clinical relevance of systematically tested antimitochondrial antibodies in systemic sclerosis.Arthritis research & therapy · 2026Article
- Novel Biomarker for Antimitochondrial Antibodies in Systemic Sclerosis Patients.Journal of immunology research · 2026Article
- Case Report: Value of MR relaxometry and spectroscopy in multimodal imaging characterization of limbic encephalitis in systemic sclerosis.Frontiers in radiology · 2026Article
- First, paradigmatic case of AMA-negative primary biliary cholangiitis with rods and rings autoantibodies: report of future implications.Gastroenterology and hepatology from bed to bench · 2025Article
- Clinical relevance and frequency of cytoplasmic patterns observed in ANA-Hep-2: experience of Cairo University Hospitals.Immunologic research · 2024Observational
- Evaluation strategy of anti-mitochondrial antibodies M2-negative: the role of multiplex rodent tissues and related clinical implications.Gastroenterology and hepatology from bed to bench · 2024Article
- Systemic sclerosis and primary biliary cholangitis share an antibody population with identical specificity.Clinical and experimental immunology · 2023Article
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8 authors at 4 institutions in 2 countries.
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Abstract
Introduction: Antimitochondrial antibodies (AMAs) are the hallmark of primary biliary cholangitis (PBC) but can be identified also in patients with connective tissue disease, namely, systemic sclerosis (SSc). Protein immunoprecipitation (IP) and IP-Western blot (WB) can be used to confirm AMA positivity directed at the pyruvate dehydrogenase complex (PDC) subunits E1α, E1β, E2/E3, and E3BP in patients showing a cytoplasmic reticular pattern at indirect immunofluorescence when performed in a screening setting before the onset of overt cholestasis in rheumatic patients. Patients and Methods: We studied sera from 285 patients affected by connective tissue disease [SSc, n = 144; dermato/polymyositis (DM/PM), n = 56; and undifferentiated connective tissue disease (UCTD), n = 85] by indirect immunofluorescence (IIF), protein-IP, and IP-WB to identify specific PDC subunits recognized by AMA. Results: Twenty percent (57/285) of sera from patients with connective tissue disease had a cytoplasmic reticular pattern at IIF, and in 77% (44/57, including 20 SSc, 12 PM/DM, and 12 UCTD) of these, we detected different titers of autoantibodies against the PDC subunits, specifically against PDC-E2. Among these sera, 4 (9%) tested positive for anti-E1α, 15 (34%) for anti-E1β, and 16 (36%) for anti-E3BP. Four of the 20 AMA-positive SSc cases (20%) had been already diagnosed with PBC, and all were positive for autoantibodies against the subunits PDC-E2, E3, and E3BP. Conclusions: Using IIF and IP, we confirm that autoantibodies against the PDC components are detected in rheumatic patients with PBC or without liver dysfunction. In view of the strong predictive value of AMA for PBC, a strict follow-up of these latter patients is warranted for an early diagnosis of the disease.
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