کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8820368 | 1609492 | 2017 | 4 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Idiopathic pleuroparenchymal fibroelastosis (PPFE) - A case study of a rare entity
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موضوعات مرتبط
علوم پزشکی و سلامت
پزشکی و دندانپزشکی
پزشکی ریوی و تنفسی
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چکیده انگلیسی
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) was recognized as a rare new entity. We report the case of a 63 years old female suffering from progressive dyspnea and dry cough for three years. Two years before admission to our hospital, idiopathic pulmonary fibrosis (IPF) was diagnosed in another hospital and treatment with prednisolone and N-acetylcysteine (NAC) was commenced. At admission HRCT showed upper lobe dominant fibrosis and associated pleural thickening. Surgical biopsies were re-evaluated and revealed fibroelastosis with pleural thickening and a probable UIP pattern, consistent with idiopathic PPFE. Treatment with pirfenidone was initiated due to progression under prednisolone and NAC. Upper lobe predominant pleural thickening with associated subpleural fibrotic changes should raise suspicion of PPFE.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Revista Portuguesa de Pneumologia (English Edition) - Volume 23, Issue 6, NovemberâDecember 2017, Pages 352-355
Journal: Revista Portuguesa de Pneumologia (English Edition) - Volume 23, Issue 6, NovemberâDecember 2017, Pages 352-355
نویسندگان
E.B. Boerner, U. Costabel, T.E. Wessendorf, D. Theegarten, F. Bonella,