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一名特发性肺纤维化患者的肺纤维溶解:吡非尼酮治疗后临床和影像学表现的改善

Pulmonary fibrolysis in a patient with idiopathic pulmonary fibrosis: improvement of clinical and radiological pattern after treatment with pirfenidone.

作者信息

Varone Francesco, Mastrobattista Annelisa, Franchi Paola, Viglietta Luca, Poletti Venerino, Tomassetti Sara, Dubini Alessandra, Tagliaboschi Linda, Calandriello Lucio, Farchione Alessandra, Larici Anna Rita

机构信息

Cardio-Thoracic Department, Pulmonology Unit, Catholic University, "A. Gemelli" Hospital, Rome, Italy.

Department of Bioimaging and Radiological Sciences, Institute of Radiology, Catholic University, "A. Gemelli" Hospital, Rome, Italy.

出版信息

Clin Respir J. 2018 Jan;12(1):347-351. doi: 10.1111/crj.12515. Epub 2016 Jul 7.

Abstract

Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia associated with the histologic and/or radiologic pattern of usual interstitial pneumonia (UIP). Nowadays, the high-resolution computed tomography pattern of "definite UIP" is enough to define a diagnosis of UIP without histological proof. This is pivotal especially in elderly patients with comorbidities. Early recognition of IPF is relevant for its prognostic implication. Some pharmacological strategies have been proposing novel molecules that tend to slow lung function decline, even though without healing fibrosis. We report a case of ex-heavy smoker male with IPF showing clinical and radiological improvement after 11 months of treatment with Pirfenidone. The definite diagnosis was challenging and it was reached by a multidisciplinary approach.

摘要

特发性肺纤维化(IPF)是一种慢性、进行性、纤维化间质性肺炎,与普通间质性肺炎(UIP)的组织学和/或放射学模式相关。如今,“明确的UIP”的高分辨率计算机断层扫描模式足以在没有组织学证据的情况下定义UIP的诊断。这一点在患有合并症的老年患者中尤为关键。IPF的早期识别因其预后意义而至关重要。一些药物策略一直在提出新的分子,这些分子倾向于减缓肺功能下降,尽管无法治愈纤维化。我们报告一例曾重度吸烟的男性IPF患者,在用吡非尼酮治疗11个月后显示出临床和放射学改善。明确诊断具有挑战性,是通过多学科方法得出的。

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