Abstract
A 54 year-old man was admitted with dry cough and exertional dyspnea. A diagnosis of idiopathic pulmonary fibrosis (IPF) was made based on his past history, chest X-ray, Gallium scintigram, bronchoalveolar lavage and TBLB specimens. Treatment with prednisolone, 80mg daily, was started, and his hypoxia, as well as chest X-ray, improved in response to the treatment. One month later, however, dyspnea suddenly developed and decreased PaO2 was noted even with 60mg prednisolone treatment daily. Pulse therapy was performed and azatioprine was also administered. Mild increase of PaO2 was noted, but it again began to decrease after a week. Double filtration plasmaphresis was therefore initiated. Immediately after the first plasmapheresis, PaO2 rose and has remained at 70torr in room air for the next seven months.
Althought the pathogenesis of IPF is not clearly understood, much evidence suggests that alveolar macrophages, activated by immune complexes (IC), play a major role in IPF. According to this hypothesis, plasmapheresis, which removes IC from plasma, should be a effective treatment for IPF. Our succesful experience supoorts this hypothesis and plasmaperesis should be tried for rapidly progressive IPF with poor prognosis, combined with conventional therapy by corticosteroid and immunosupressive agents.
