Rheumatic disorders and primary biliary cirrhosis: an appraisal of 170 Italian patients

Objective-To establish the frequency of connective tissue diseases (CTD) in a cohort of Italian patients with primary biliary cirrhosis (PBC) and to e valuate the availability of a marker for the early identification of the mo re common associated CTD. Methods-One hundred and seventy consecutive patients with histologically di agnosed PBC were screened for the presence of a CTD and/or Raynaud's phenom enon (RP). Patients were classified as having a CTD only if they fulfilled standardised criteria. Results-Forty seven patients had a CTD. The most common CTD was systemic sc lerosis (SSc), found in 21 patients. RP was present in 54 patients, most of whom (n=39) had an associated CTD. The most prevalent autoantibodies inclu ded antinuclear antibodies (ANA) with anticentromere (ACA) and speckled pat terns (34 and 33 patients, respectively) and extractable nuclear antigens ( ENA, 27 patients). However, while the frequencies of ACA and ENA were signi ficantly higher in patients with an associated CTD (p <0.0001 and p <0.005, respectively), no relationship was found for speckled ANA. ACA was the bes t predictor of a CTD in patients with PBC (odds ratio (OR) 24.5, 95% CI 5.5 to 108.8), followed by the presence of ENA (OR 23.9, 95% CI 5.6 to 101.0) and RP (OR 20.2, 95% CI 5.7 to 71.2). Conclusions-Using strict standardised classification criteria we have found that SSc is the most common CTD associated with PBC and that ACA and ENA a re strong markers for an associated CTD in patients with PBC.