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Primary biliary cirrhosis: lung involvement

C Costa, A Sambataro, S Baldi

    Liver
    |August 1, 1995
    PubMed
    Summary
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    Sub-clinical lung impairment, specifically reduced alveolar gas diffusion, is common in primary biliary cirrhosis. This lung function abnormality is linked to CREST syndrome and anti-centromere antibodies, not liver disease severity.

    Area of Science:

    • Pulmonology
    • Hepatology
    • Rheumatology

    Background:

    • Sub-clinical lung impairment, characterized by reduced alveolar gas diffusion, is a known complication of advanced primary biliary cirrhosis.
    • Pulmonary involvement in primary biliary cirrhosis often occurs without respiratory symptoms.

    Purpose of the Study:

    • To determine the prevalence and nature of lung involvement in primary biliary cirrhosis.
    • To investigate the relationship between lung function abnormalities and clinical/epidemiological factors in primary biliary cirrhosis patients.

    Main Methods:

    • Evaluated 61 primary biliary cirrhosis patients using spirometry and diffusing capacity for carbon monoxide (DLCO) measurements.
    • Assessed liver disease advancement via histological stage, portal hypertension signs, and Mayo Risk Score.

    Related Experiment Videos

  • Collected data on smoking habits, rheumatological disorders (including CREST syndrome and Sjogren syndrome), and autoantibodies.

    • Main Results:

    • Reduced alveolar diffusion capacity (DLCO) was found in 39% of patients, with no respiratory symptoms.
    • No significant correlation was observed between DLCO and smoking, liver disease stage, or Sjogren syndrome.
    • Reduced DLCO significantly correlated with CREST syndrome (p < 0.01) and anti-centromere antibodies (p < 0.05).

    Conclusions:

    • Alveolar diffusion capacity is frequently impaired in primary biliary cirrhosis patients, often asymptomatically.
    • This impairment is significantly associated with concomitant CREST syndrome and the presence of anti-centromere antibodies.