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Primary biliary cholangitis, previously known as primary biliary cirrhosis (PBC), is the most common autoimmune diseases of the liver (ALD). Patients with PBC may present with typical biliary pattern symptoms. Also the presence of anti-mitochondrial autoantibodies (AMAs) is the laboratory hallmark of PBC, which molecular target antigens are members of 2-oxoacid dehydrogenase complex of enzymes. In recent years, autoantibodies (Aab) targeting subcellular structures described as the rods and rings (R&R) pattern in HEp-2 ANA have been presented as a unique and particular case of Aab generation. These R&R structures are composed of inosine monophosphate dehydrogenase type 2 (IMPDH2), and their formation can be induced in vitro by several small-molecule inhibitors. Aab targeting these relatively unknown structures have been almost exclusively observed in hepatitis C virus (HCV) patients who have undergone treatment with pegylated interferon-a/ribavirin (IFN/RBV) therapy. Literature showed that anti-RR Aab have not been found in no treatment-naïve HCV patients or in patients from any other disease. Now we present and characterized a case patient with contemporary presence of AMAs and R&R Aab in PBC, without any laboratory evidence of HCV and/or other hepatic virus infection. For our knowledge, this is the first case described in the Literature. R&R Aab in patients without any clinical/laboratory signs or symptoms of Hepatitis virus and without pharmacological therapy open the window to the alternative scenario: the association of these Aab to ALD. Our work demonstrated that R&R Aab can be present in PBC case. The interesting idea suggested that R&R Aab may be present also in AMA-negative PBC and they can considered a new possible diagnostic tool in this specific clinical condition. Other study and cases are needed but the presence of R&R Aab linked with AMAs and PBC may be explained by alterations in immune regulation caused by autoimmunity in a particular genetic background.

Primary biliary cholangitis with contemporary presence of anti-mithocondrial and anti-rods and rings autoantibodies: literature first case