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This report presents the case of a patient with corticosteroid and cyclophosphamide resistant exacerbation of idiopathic pulmonary fibrosis (IPF), which was definitely resolved in accordance with increased levels of serum conjugated bilirubin due to biliary tract obstruction. Histological examination of the lung showed an accumulation of bile pigments in the alveolar mural 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 inflammatory damage in IPF, and may illustrate an important key for treatment of this fatal disorder.

A Patient with Exacerbation of Idiopathic Pulmonary Fibrosis Which was Resolved Probably due to the Coexisting Hyperbilirubinemia?