RT Journal Article SR Electronic T1 Primary Biliary Cirrhosis-related Autoantibodies in a Large Cohort of Italian Patients with Systemic Sclerosis JF The Journal of Rheumatology JO J Rheumatol FD The Journal of Rheumatology SP jrheum.110167 DO 10.3899/jrheum.110167 A1 Ilaria Cavazzana A1 Angela Ceribelli A1 Mara Taraborelli A1 Micaela Fredi A1 Gary Norman A1 Angela Tincani A1 Minoru Satoh A1 Franco Franceschini YR 2011 UL http://www.jrheum.org/content/early/2011/09/12/jrheum.110167.abstract AB 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.