Primary biliary cirrhosis-related autoantibodies in a large cohort of italian patients with systemic sclerosis.

Abstract

OBJECTIVE:

To analyze the prevalence, associations, and fine specificity of autoantibodies to primary biliary cirrhosis (PBC)-associated antigens (MIT3, Sp100, and gp210) in a cohort of Italian patients with systemic sclerosis (SSc).

METHODS:

Sera samples from 201 patients with SSc were tested for antibodies to MIT3, gp210, and Sp100 by ELISA (the PBC screen). Anti-MIT3-positive sera were studied for IgG or IgA isotypes. All sera were analyzed by indirect immunofluorescence on HEp-2 cells and on rodent kidney/stomach/liver tissue sections in order to detect antinuclear and antimitochondrial antibodies (AMA). SSc was selected by American College of Rheumatology criteria and classified based on LeRoy's criteria.

RESULTS:

Forty-three (21.4%) sera samples were positive for PBC screen antibodies. Anti-MIT3 antibodies were detected in 36 samples, anti-Sp100 in 5, and anti-gp210 in 1 sample. The other 3 PBC screen-positive samples showed no specificity for the single antigens. PBC screen-positive patients more frequently showed a limited cutaneous SSc subtype (p = 0.04), anticentromere antibodies (ACA; p = 0.0013), elevated alkaline phosphatase (ALP) (p < 0.0001), PBC (p = 0.002), and AMA (p = 0.008). Teleangiectasia and calcinosis were less frequent in this group of patients. IgG+IgA anti-MIT3-positive patients had higher prevalence of AMA (p = 0.0035), diagnosis of PBC (p = 0.014), and increased ALP (p = 0.039), all considered biochemical markers of severe liver disease.

CONCLUSION:

PBC screen antibodies were detected in 20% of patients with SSc, strongly associated with ACA. ACA+/PBC screen+ patients had higher risk of developing PBC or elevation of ALP.

• Authors: Ilaria Cavazzana, Angela Ceribelli, Mara Taraborelli, Micaela Fredi, Gary Norman, Angela Tincani, Minoru Satoh, Franco Franceschini
• Journal: The Journal of rheumatology (J Rheumatol) Vol. 38 Issue 10 Pg. 2180-5 (Oct 2011) ISSN: 0315-162X [Print] Canada
• PMID: 21921093 (Publication Type: Journal Article)

Chemical References

• Antigens, Nuclear
• Autoantibodies
• Autoantigens
• MIT3 fusion protein
• NUP210 protein, human
• Nuclear Pore Complex Proteins
• Recombinant Fusion Proteins
• SP100 protein, human

Topics

• Adult
• Aged
• Antigens, Nuclear (immunology)
• Autoantibodies (immunology)
• Autoantigens (immunology)
• Female
• Humans
• Italy
• Liver Cirrhosis, Biliary (diagnosis, immunology)
• Male
• Middle Aged
• Nuclear Pore Complex Proteins (immunology)
• Predictive Value of Tests
• Recombinant Fusion Proteins (immunology)
• Scleroderma, Systemic (immunology)
• Sensitivity and Specificity