Background Primary biliary cirrhosis (PBC) is frequently associated with connective tissue diseases, namely systemic sclerosis (SSc). Predictive factors for survival of patients with both PBC and SSc are controversial. In 2005, we started a prospective study of 100 PBC patients to determine the prevalence of SSc, to study the clinical, biological and microvascular aspects of patients that had both diseases, and to identify predictive factors for progression of hepatic disease. Results of first visit for 23 patients with PBC and SSc (PBC-SSc) and 23 patients with PBC only were presented during the 2008 EULAR congress.
Objectives to report the clinical, biological and microvascular features of PBC patients at first follow-up visit.
Methods The medical history, physical examination, biological investigations and quantitative nailfold capillary microscopy (NMC) were repeated at follow-up visit.
Results 90 patients were available for a second visit after a mean follow-up of 3.05 years. Among them 19 had PBC only and SSc was present in 22 (23%) patients (19 limited cutaneous SSc and 3 early SSc). Signs of portal hypertension or hepatic failure were present in 4 (9.7%) patients. In PBC-SSc patients, a typical NCM SSc pattern was observed in 17 (77%) patients and was identical at follow-up visit. No patient with CBP only developed abnormal NCM at follow-up study. The Mayo score was similar in patients with vs without SSc and no signifcant difference was observed for level of Alkaline Phosphatase in patients with versus without SSc.
Conclusions Presence of SSc did not worsen the evolution of PBC patients at first follow-up visit. In patients with SSc, the cutaneous and NCM patterns are stable. Long term follow-up is still ongoing to survey the occurrence of SSc associated complications, namely pulmonary hypertension and fibrosis.
Disclosure of Interest None Declared
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