Autoantibodies against integral membrane proteins of the nuclear envelope in patients with primary biliary cirrhosis
Abstract
Background/aims Autoantibodies against nuclear membrane proteins have been identified in patients with primary biliary cirrhosis (PBC). The aim of the present study was to determine the incidence of these autoantibodies in patients with PBC and examine their significance. Methods An assay using recombinant polypeptides was designed to unequivocally detect autoantibodies against gp210 and the lamin B receptor, integral proteins of the nuclear membranes. Results Autoantibodies against gp210 were detected in 15 of 159 patients with PBC and 0 of 46 controls. Autoantibodies against lamin B receptor were detected in 2 patients with PBC and 0 controls. The presence of these autoantibodies had a sensitivity of 11% and specificity of 100% for the diagnosis of PBC. Autoantibodies against gp210 were present in 4 of 19 (21%) patients with PBC who did not have detectable antimitochondrial antibodies. Patients with PBC and gp210 autoantibodies had a higher incidence of associated arthritis. Conclusions Autoantibodies against gp210 and the lamin B receptor are present in approximately 10% of patients with PBC. These autoantibodies are highly specific for the diagnosis of PBC and may be useful in diagnosing individuals without antimitochondrial antibodies and in identifying a subgroup of patients with an increased incidence of associated arthritis.
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They are present in ~95% (mitochondrial) and ~30% (nuclear) of patients.
“They are present in ~95% (mitochondrial) and ~30% (nuclear) of patients.”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [73]
Unlike in many other autoimmune diseases, these autoantibodies are, as a result of their sensitivity and specificity, extremely useful in diagnosis and have contributed significantly to the decline in the need for liver biopsy, at least for the purposes of diagnosis.
“Unlike in many other autoimmune diseases, these autoantibodies are, as a result of their sensitivity and specificity, extremely useful in diagnosis and have contributed significantly to the decline in the need for liver biopsy, at least for the purposes of diagnosis.”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [73]
PBC is characterised serologically by autoantibodies specific for mitochondrial, nuclear and centromere antigens, some of which are unique to PBC.
“PBC is characterised serologically by autoantibodies specific for mitochondrial, nuclear and centromere antigens, some of which are unique to PBC.66–78”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [73]
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- Authors
- Nickowitz, R. E., Wozniak, R. W., Schaffner, F., & Worman, H. J.
- Affiliations
- Rockefeller University (US)
- Venue
- Gastroenterology
- Publisher
- Elsevier BV
- Published
- 1994-01
- Volume
- 106
- Issue
- 1
- Pages
- 193-199
- ISSN
- 0016-5085 (ISSN-L)
- OpenAlex
- W1832430407
- Cited by
- 96
- References
- 45
- Citations by year
- 2023: 3 · 2020: 1 · 2018: 2 · 2016: 1 · 2015: 1 · 2014: 2 · 2013: 2 · 2012: 4
- FWCI
- 5.33
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Gastroenterology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
- Keywords
- primary biliary cirrhosis, autoantibodies, gp210, lamin B receptor, nuclear envelope proteins, antinuclear antibodies, antimitochondrial antibodies, sensitivity and specificity, arthritis
- MeSH terms
- Lamin B Receptor, Autoantibodies, Fluorescent Antibody Technique, Humans, Liver Cirrhosis, Biliary, Membrane Glycoproteins, Membrane Proteins, Nuclear Envelope, Nuclear Proteins, Recombinant Proteins, Sensitivity and Specificity, Receptors, Cytoplasmic and Nuclear, Nuclear Pore Complex Proteins
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