Primary Biliary Cholangitis in Elderly: About 12 Tunisian Cases

Lamine, Zeineb Ben; Mankaï, Amani; Ahmed, M.; Jazia, I. Ben; Slama, A. Ben; Baccouhe, Azza; Jemaa, A.; Ghedira, Ibtissem

doi:10.4172/2155-9899.1000564

Cited by 2 publications

(1 citation statement)

References 18 publications

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“…PML was originally identified as a protein aberrantly expressed in leukemic cells of patients with acute promyelocytic leukemia. Based on different studies, anti-Sp100 and anti-PML autoantibodies can be detected in 20–30% of patients with PBC [ 7 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 ]. The co-localization of Sp100 and PML in nuclear dots suggests association of both proteins in multi-subunit complexes.…”

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confidence: 99%

Analysis of Autoantibodies against Promyelocytic Leukemia Nuclear Body Components and Biochemical Parameters in Sera of Patients with Primary Biliary Cholangitis

et al. 2021

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Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by immune-mediated destruction of intrahepatic bile ducts and the presence of specific antibodies. The aim of the study was to examine the diagnostic significance of antibodies against promyelocytic leukemia nuclear body (PML NB) components such as Sp100, Sp140, and PML in a cohort of PBC patients and compare the results with biochemical and histological parameters. Serum samples were collected from 93 PBC patients. Anti-Sp100 and anti-PML antibodies were assessed using commercially available kits, anti-Sp140 using developed “in-house” ELISA test. Anti-Sp140, anti-Sp100, and anti-PML antibodies were present in 25 (27%), 37 (40%), and 29 (31%) PBC patients, respectively. Anti-PML NB positive patients also showed increased concentration of bilirubin and alkaline phosphatase (p < 0.05). In the group with the presence of at least two types of these antibodies, more frequent deaths or transplantations were observed. A correlation between the presence of antibodies and histological grade (OR = 2.55 p = 0.039) was established. Patients with bilirubin > 1.1 mg/dL at the time of diagnosis had a significantly shorter time of survival than patients with bilirubin ≤ 1.1 mg/dL (HR 5.7; 95% C.I., 2.7, 12.3; p < 0.001). Our data confirm very high specificity of anti-PML NB antibodies, which can expand the laboratory diagnostic capabilities of PBC. We found an association between positive reactivity of autoantibodies directed against components of PML nuclear bodies and higher concentrations of bilirubin and alkaline phosphatase, but the main prognostic marker of survival remains serum bilirubin.

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