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Re: Prognosis of Primary Biliary Cirrhosis in Japanese Systemic Sclerosis Patients

Dear Editor,

We would like to add our observations to those of Rigamonti et al. regarding the prognosis of 43 primary biliary cirrhosis (PBC) in systemic sclerosis (SSc) patients (observation period: average 3.1 years)(Gut 2005;55:388). Previously, we reported that PBC-SSc patients have additional antibodies to E1-alpha epitope besides the commonly observed E2 epitope in primary PBC, revealing mild liver dysfunction in these patients1. Since PBC is a life threatening disease and the prevalence of PBC in autoimmune diseases is the highest in SSc, it is very important to know the long-term prognosis of liver dysfunction in PBC-SSc patients.

In our study, we retrospectively evaluated the development of liver dysfunction in 75 anti-centromere antibody (ACA) positive Japanese SSc patients whom we could follow for 5 years or longer (up to 15 years). No patients developed severe liver dysfunction whom we could not follow up more than 5 years. We found out 16 patients (21.3%) positive for anti-mitochondrial antibody (AMA) out of 75 ACA positive SSc patients. Eleven of 16 patients (68.8%) had elevated serum alkaline phosphatase (ALP) levels. Other potential causes of the liver enzyme abnormalities in these patients including alcoholism, infection by hepatitis B or C virus, drug allergies, and right ventricular failure, were excluded by careful inquiry and laboratory examinations. All 11 SSc patients who were diagnosed as PBC were asymptomatic for PBC at first visit. We compared the ratio of serum ALP levels at first and latest visit in PBC-SSc patients to evaluate alterations of liver function. The ratio of ALP (latest visit / first visit) was below 1.5 in all 11 patients. There were no patients who developed symptomatic PBC during observation. Although two AMA-positive SSc patients with normal ALP levels at their first visit developed elevated ALP levels later, both patients showed only slight liver dysfunction. Our observation revealed favorable prognosis of PBC in Japanese patients with SSc. The immunological effects from SSc may affect favorable prognosis in PBC-SSc patients.

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