Usual interstitial pneumonia associated with primary biliary cirrhosis

SummaryWe herein describe the first case, to our knowledge, of surgical biopsy-proven usual interstitial pneumonia (UIP) as the pulmonary manifestation in primary biliary cirrhosis (PBC). A 56-year-old male was admitted to our hospital because of the identification of bilateral reticular infiltrate on a chest roentgenogram. He was histologically confirmed to have PBC without definite signs of Sjögren's syndrome at age 47. His chest CT scan showed bibasilar reticular opacities without a honeycomb appearance, while a bronchoalveolar lavage fluid analysis revealed no lymphocytosis. A surgical lung biopsy specimen revealed evidence of a UIP pattern. The patient's condition has remained stable for a follow-up period of two years without treatment. Our case indicates that the UIP pattern is an additional form of diffuse interstitial pneumonia/fibrosis that may develop in association with sporadic PBC