Antinuclear antibodies as ancillary markers in primary biliary cirrhosis

  title={Antinuclear antibodies as ancillary markers in primary biliary cirrhosis},
  author={Alessandro Granito and Paolo Muratori and Chiara Quarneti and Georgios Pappas and Ronny Cicola and Luigi Muratori},
  journal={Expert Review of Molecular Diagnostics},
  pages={65 - 74}
Antimitochondrial antibodies are the serological hallmark of primary biliary cirrhosis (PBC). Besides antimitochondrial antibodies, the autoantibody profile of PBC includes antinuclear antibodies (ANA) which are detectable by indirect immunofluorescence in up to 50% of PBC patients. Two immunofluorescence patterns are considered ‘PBC-specific’: the multiple nuclear dots and rim-like/membranous patterns. The target antigens of the multiple nuclear dots pattern have been identified as Sp100 and… 
The diagnosis of antimitochondrial antibody-negative primary biliary cholangitis.
Clinical significance of autoantibodies in primary biliary cirrhosis.
Because the natural course of PBC is being altered by treatment with ursodeoxycholic acid, the clinical significance of these PBC-specific autoantibodies awaits re-evaluation in various ethnicities.
Analysis of Autoantibodies against Promyelocytic Leukemia Nuclear Body Components and Biochemical Parameters in Sera of Patients with Primary Biliary Cholangitis
Very high specificity of anti-PML NB antibodies are confirmed, which can expand the laboratory diagnostic capabilities of PBC, and an association between positive reactivity of autoantibodies directed against components of PML nuclear bodies and higher concentrations of bilirubin and alkaline phosphatase is found, but the main prognostic marker of survival remains serum bilirube.
Detection of Autoantibodies Against Nucleoporin p62 in Sera of Patients With Primary Biliary Cholangitis
Anti-p62 may be regarded as a significant serological marker of PBC and the sensitivity, specificity, positive and negative predictive values (PPV and NPV), and positive andnegative likelihood ratio (LR+ and LR−) of the in-house ELISA for diagnosing PBC based on anti-p 62 were calculated.
Clinical significance of the fluctuation of primary biliary cirrhosis-related autoantibodies during the course of the disease
It is suggested that detection of AMA and PBC-specific ANA was correlated with disease severity andSerial changes of anti-sp100 titers and not ofAnti-gp210 titers might prove useful for monitoring the disease course and treatment outcome.
Autoantibodies to speckled protein family in primary biliary cholangitis
The autoantibody profile of primary biliary cholangitis includes antinuclear antibodies (ANA) which are detectable by indirect immunofluorescence in more than 50% of PBC patients, suggesting novel and more refined therapeutic approaches.
Antinuclear Antibodies: Marker of Diagnosis and Evolution in Autoimmune Diseases
Depending on the subtype of ANA present in the serum and the targeted antigen, several staining patterns are reported, namely, nuclear patterns, nucleolar patterns, cell cycle patterns, or cytoplasmatic patterns, which can lead to diagnosis of a certain autoimmune disease.
Primary biliary cholangitis with contemporary presence of anti-mithocondrial and anti-rods and rings autoantibodies: literature first case
  • R. Assandri
  • Medicine, Biology
    Gastroenterology and hepatology from bed to bench
  • 2019
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.
Promyelocytic Leukemia Antigen Expression: a Histological Marker for Primary Biliary Cholangitis Diagnosis?
It is postulate that a simple PML immunohistochemical test could be sufficient for histopathological discrimination of PBC in problematic cases of undefined cholestatic disorders, including small-duct PSC and AMA-negative PBC cases.


Specificity of anti‐sp100 antibody for primary biliary cirrhosis
Though the regional differences on anti-sp100 antibody described above may be explained by different environments or different genetic backgrounds, it is important that further studies conducted on large cohorts of patients and in different geographic areas are undertaken to resolve the question of the sensitivity and specificity of anti- sp100 for PBC.
Anti‐sp100 antibodies in primary biliary cirrhosis
It is suggested that anti-sp100 ELISA is more sensitive than IFL in detecting disease-specific ANA reactivities and has to be complemented or replaced with molecular-based techniques that discriminate among defined nuclear antigens.
Specificity of antinuclear antibodies in primary biliary cirrhosis.
The high frequencies of various antibodies directed against intracellular proteins and nucleic acids in patients with PBC suggests that PBC is a multisystem autoimmune disease which is similar to other systemic autoimmune diseases.
Prevalence and clinical significance of isotype specific antinuclear antibodies in primary biliary cirrhosis
PBC specific ANA, in particular of the IgG3 isotype, are associated with a more severe disease course, possibly reflecting the peculiar ability of this isotype to engage mediators of damage.
PML Nuclear Body Component Sp140 Is a Novel Autoantigen in Primary Biliary Cirrhosis
The very frequent coexistence ofanti-Sp140, anti-Sp100 and anti-PML antibodies suggests that the NB is a multiantigenic complex in PBC and enhances the diagnostic significance of these reactivities, which are particularly useful in AMA-negative cases.
Autoantibodies in primary biliary cirrhosis
The analysis of the clinical relevance of the four different AMA profiles revealed that they correlate with the two different courses of PBC, suggesting the presence of a stimulatory agent as well as a B cell defect in PBC patients, which is defined by the lack of the production of NOMA.
Clinical significance of anti-multiple nuclear dots/Sp100 autoantibodies.
Testing for MND/Sp100 positivity is useful for the diagnosis of PBC, but only when the right clinical context is present and other diseases cannot be excluded in first line SLE.