TY - JOUR T1 - Primary Biliary Cirrhosis-related Autoantibodies in a Large Cohort of Italian Patients with Systemic Sclerosis JF - The Journal of Rheumatology JO - J Rheumatol DO - 10.3899/jrheum.110167 SP - jrheum.110167 AU - Ilaria Cavazzana AU - Angela Ceribelli AU - Mara Taraborelli AU - Micaela Fredi AU - Gary Norman AU - Angela Tincani AU - Minoru Satoh AU - Franco Franceschini Y1 - 2011/09/15 UR - http://www.jrheum.org/content/early/2011/09/12/jrheum.110167.abstract N2 - 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. ER -