The study of pathogenesis of rapidly progressive interstitial pneumonia associated with polymyositis/dermatomyositis and its novel treatments

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Intratracheal injection of bleomycin 2 mg/kg (low dose) and 10 mg/kg (high dose) induced fibrotic lung lesions and hemorrhagic lung lesions, respectively. Thus, mice models of rapidly progressive interstitial pneumonia have been established.Moreover, a 3-dimensional mixed culture of human lung epithelial cells and human pulmonary fibroblasts was used as a screening test for novel therapeutic agents for rapidly progressive interstitial pneumonia. Imatinib, a drug for chronic myeloid leukemia, selectively inhibited the proliferation of pulmonary fibroblasts stimulated by platelet-derived growth factor.These results elucidated the possible treatment of rapidly progressive interstitial pneumonia by targeted therapies.