Background & Aim. Primary biliary cirrhosis (PBC) is frequently associated with other autoimmune diseases, including systemic sclerosis (SSc). In the last years many efforts have been dedicated to the research of widely accepted criteria for the early diagnosis of SSc. Since studies on the prevalence of early SSc in PBC patients are lacking, our aim was to investigate its hitherto unknown prevalence in a large cohort of PBC patients.

. We studied 80 PBC patients and 72 patients with other chronic liver diseases. Diagnostic workup included research into signs of connective tissue disease, determination of autoantibody profile, and examination of capillary abnormalities through nailfold videocapillaroscopy.

. Ten PBC patients (12.5%) satisfied diagnostic criteria for early SSc and 5 (6.3%) had definite SSc. None of the patients in the control group were diagnosed either with early or definite SSc. No differences were observed in terms of aminotransferases, alkaline phosphatase, and liver function tests between PBC patients with and without associated SSc. 

. Early SSc is significantly frequent in PBC patients. The detection of early SSc in PBC patients may lead to a prompt treatment of its complications, preventing inabilities and preserving the chance of liver transplantation.

: ACA: anti-centromere antibodies; ACR: American College of Rheumatology; AMA: antimitochondrial antibodies; ANA: anti-nuclear antibodies; CENP-B: Centromere-protein B; ENA: extractable nuclear antigens; IIF: indirect immunofluorescence; NCM: nailfold capillary microscopy; PBC: Primary biliary cirrhosis; RP: Raynaud's phenomenon; SSc: Systemic sclerosis; UDCA: ursodeoxycholic acid.


primary biliary cirrhosis, systemic sclerosis, Raynaud phenomenon, nailfold capillaroscopy, anti-centromere antibodies