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_11
Snippet: In this study, we described two series of familial cases of interstitial lung disease of unknown etiology, in a young male patient and his father, and two young girls and their mother. They presented with the same clinical symptoms, showed rapidly progressive respiratory failure of unknown cause, and had similar radiological and pathological features, including rapidly prohttp://e-aair.org gressive lung fibrosis. As this disease has not been prev.....
Document: In this study, we described two series of familial cases of interstitial lung disease of unknown etiology, in a young male patient and his father, and two young girls and their mother. They presented with the same clinical symptoms, showed rapidly progressive respiratory failure of unknown cause, and had similar radiological and pathological features, including rapidly prohttp://e-aair.org gressive lung fibrosis. As this disease has not been previously described, we named this disease entity 'unclassified interstitial pneumonia with fibrosis. ' Previously, we reported this unclassified interstitial pneumonia with rapidly progressive pulmonary fibrosis as AIP. 2, 3 AIP is a potentially fatal respiratory disease of unknown etiology that presents in previously healthy individuals. [4] [5] [6] [7] [8] Unclassified interstitial pneumonia with fibrosis, which is not classified as any of the disease entities in the classification of interstitial lung diseases in adults, 1 shows clinical and radiological findings that are similar to those of AIP. However, these two diseases have different pathological findings. Unclassified interstitial pneumonia with fibrosis is characterized histologically by centrilobular distribution of alveolar damage and bronchiolar destruction with bronchiolar obliteration.
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