A patient with exacerbation of idiopathic pulmonary fibrosis which was resolved probably due to the coexisting hyperbilirubinemia?

This report presents the case of a patient with corticosteroid and cyclopho sphamide resistant exacerbation of idiopathic pulmonary fibrosis (IPF), ml lich was definitely resolved in accordance with increased levels of serum c onjugated bilirubin due to biliary tract obstruction. Histological examinat ion of the lung showed an accumulation of bile pigments in the alveolar mur al tissues, especially in the cytoplasm of the alveolar macrophages, which play crucial roles in the development of IPF. This case suggests that bile pigments have some important roles in tissue protection against inflammator y damage in IPF, and may illustrate an important key for treatment of this fatal disorder.