First, paradigmatic case of AMA-negative Primary bilairy cholangiitis with Rods and Rings autoantibodies: report of future implications
Gastroenterology and Hepatology from Bed to Bench,
Vol. 18 No. 3 (2025),
26 August 2025
https://doi.org/10.22037/ghfbb.v18i3.3135
Abstract
Primary biliary cholangitis (PBC) is the most common autoimmune diseases of the liver. Patients with PBC in characterised by typical biliary pattern symptoms and the presence of anti-mitochondrial autoantibodies (AMAs) in more than 90% of patients and around 50% also show antinuclear antibodies (ANAs).
Based on different studies, anti-Sp100 and anti-PML autoantibodies can
be detected in 20–30% of patients with PBC, mostly in AMA-negative PCB. In recent years, autoantibodies (Aab) targeting subcellular structures described as the Rods and Rings (R&R) have been presented as a unique and particular case of Aab generation.
Now we characterized a patient with contemporary presence of Sp100, PML and R&R Aab (AC-23 pattern following ICAP nomenclature) in PBC, without any laboratory evidence of HCV and/or other hepatic virus infection. For our knowledge, this is the first case of AMA-negative PCB with R&R Aab described in the Literature.
Review of the Literature showed that the AC-23 pattern are present not only in HCV-patient in interferon-ribavirin treatment but, also in ALD.
R&R Aab in patients with PCB confirm and reinforce the association of these Aab to ALD.
- Autoimmune Hepatitis, Primary Sclerosing Cholangitis, Inflammatory Bowel Disease, Ulcerative Colitis, overlap syndrome.
How to Cite
References
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