Author: Lee, Eun; Seo, Ju-Hee; Kim, Hyoung-Young; Yu, Jinho; Song, Jin Woo; Park, Young Soo; Jang, Se-Jin; Do, Kyung-Hyun; Kwon, Jiwon; Park, Sung-woo; Park, Jeong-hwan; Hong, Soo-Jong
Title: Two Series of Familial Cases With Unclassified Interstitial Pneumonia With Fibrosis Document date: 2012_3_9
ID: pwlcqavv_16
Snippet: Although there is no proven effective therapy for unclassified interstitial pneumonia with fibrosis, recommended treatments include supportive care and immunosuppressive therapies such as corticosteroids, cyclophosphamide, and vincristine. 7,10 These treatments were found to be most effective during the early proliferative phase, i.e., before the development of fibrosis in the late fibrotic phase. 9 Early treatment with corticosteroids, cyclophos.....
Document: Although there is no proven effective therapy for unclassified interstitial pneumonia with fibrosis, recommended treatments include supportive care and immunosuppressive therapies such as corticosteroids, cyclophosphamide, and vincristine. 7,10 These treatments were found to be most effective during the early proliferative phase, i.e., before the development of fibrosis in the late fibrotic phase. 9 Early treatment with corticosteroids, cyclophosphamide, and hydroxychloroquine, a combination designed to inhibit fibrogenesis, might have contributed to the more favorable outcome in Case 1 of the present series. In addition, the presence of a pneumothorax and/or a pneumomediastinum on chest radiography (case 3) and the severity of the respiratory difficulty, both reflecting disease severity, may predict poor response to treatment.
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