Objective: To summarize the clinical characters of refractory primary biliary cirrhosis (PBC) versus typical ones and explore the risk factors of prognosis.
Methods: From 2009 to 2011, 60 PBC patients on the therapy of ursodeoxycholic acid (UDCA) over 1 year at our clinic were recruited. According to the response to UDCA by the Paris criteria, they were divided into refractory group (n = 23) and typical group (n = 37) to analyze the basic clinical conditions, biochemical markers, antibodies and liver biopsies.
Results: (1) Compared with the typical patients, the refractory ones had more fatigue (73.9% vs 45.9%), pruritus (60.9% vs 40.5%) and jaundice (43.4% vs 18.9%); (2) the serum level of biomarkers (alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), γ-glutamyl transpeptidase (GGT), total bilirubin (TBIL), direct bilirubin (DBIL) and immunoglobulin M (IgM)) were higher in the refractory group (P < 0.05); (3) no differences existed between two groups in age, gender, antibodies (antinuclear antibody (ANA), antimitochondrial antibody (AMA), AMA-M2, anti-GP210, anti-SP100 and anti-centromere antibody (ACA)) or liver biopsies.
Conclusion: Some clinical severe manifestation (fatigue, pruritus and jaundice) and higher biochemical markers levels (ALT, AST, ALP, GGT, TBIL, DBIL and IgM) seem to predict a worse response to UDCA and a rapid progression of disease in PBC patients.