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Pathologic separation of idiopathic pulmonary fibrosis from fibrotic hypersensitivity pneumonitis.

特发性肺纤维化与纤维化过敏性肺炎的病理分离。

  • 影响因子:6.13
  • DOI:10.1038/s41379-019-0389-3
  • 作者列表:"Wright JL","Churg A","Hague CJ","Wong A","Ryerson CJ
  • 发表时间:2020-04-01
Abstract

:Accurate separation of idiopathic pulmonary fibrosis from fibrotic (chronic) hypersensitivity pneumonitis is crucial to patient management, but is frequently a difficult problem. Our objective was to identify pathologic variables that help make this separation. Clinical, radiological, and pathologic data were re-reviewed for 23 patients with a fibrotic interstitial lung disease and biopsy suggesting idiopathic pulmonary fibrosis or fibrotic hypersensitivity pneumonitis. Clinical features, high-resolution computed tomography, and surgical lung biopsies were each examined independently using a prespecified approach. This was followed by a multidisciplinary discussion in which the likelihood of an idiopathic pulmonary fibrosis diagnosis was assigned by the clinician alone based only on clinical data, by the clinician and radiologist based on integrated clinical and radiologic data, and by the clinician, radiologist, and pathologist based on all three domains. A higher multidisciplinary discussion-based confidence of idiopathic pulmonary fibrosis was associated with older age at diagnosis, male sex, higher forced vital capacity, and absence of ground glass changes. Pathologic variables associated with a higher multidisciplinary discussion-based confidence of idiopathic pulmonary fibrosis included increased number of fibroblast foci/cm2 and increased subpleural fibrosis. Pathologic variables associated with a higher multidisciplinary discussion-based confidence of hypersensitivity pneumonitis included an increased fraction of bronchioles with peribronchiolar metaplasia, increased foci of peribronchiolar metaplasia/cm2, and presence of giant cells/granulomas. These results provide guidance in separating idiopathic pulmonary fibrosis from hypersensitivity pneumonitis; however, a third of cases could not be confidently classified even when using these pathologic features combined with clinical and radiologic information in a multidisciplinary discussion.

摘要

: 特发性肺纤维化与纤维化 (慢性) 过敏性肺炎的准确分离对患者管理至关重要,但往往是一个难题。我们的目的是确定有助于分离的病理变量。对 23 例纤维化间质性肺疾病患者的临床、影像学和病理资料进行了重新回顾,活检提示特发性肺纤维化或纤维化过敏性肺炎。使用预先设定的方法独立检查临床特征、高分辨率计算机断层扫描和外科肺活检。随后进行了多学科讨论,其中特发性肺纤维化诊断的可能性仅由临床医生根据临床数据进行分配,由临床医生和放射科医生基于综合临床和放射学数据,由临床医生、放射科医生和病理学家基于所有三个领域。基于多学科讨论的特发性肺纤维化置信度较高与诊断时的年龄较大、男性、较高的用力肺活量和没有磨玻璃改变相关。与特发性肺纤维化更高的多学科讨论相关的病理变量包括成纤维细胞灶/cm2 数量增加和胸膜下纤维化增加。与基于更高多学科讨论的过敏性肺炎置信度相关的病理变量包括细支气管伴细支气管周围化生部分增加,细支气管周围化生病灶增加/cm2,以及存在巨细胞/肉芽肿。这些结果为分离特发性肺纤维化和过敏性肺炎提供了指导; 然而,三分之一的病例即使在多学科讨论中使用这些病理特征结合临床和放射学信息,也无法自信地进行分类。

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METHODS:OBJECTIVES:To asses the clinical course in RA-related interstitial lung disease (RA-ILD) patients with and without rituximab (RTX). The influence of other variables was also evaluated. METHODS:A longitudinal multicentre study was conducted in RA diagnosed with ILD from 2007 until 2018 in Madrid. Patients were included in a registry [pNEumology RhEumatology Autoinmune diseases (NEREA)] from the time of ILD diagnosis. The main endpoint was functional respiratory impairment (FI), when there was a decline ≥5% in the predicted forced vital capacity compared with the previous one. Pulmonary function was measured at baseline and in follow-up visits every 6-12 months. The independent variable was therapy with RTX. Covariables included sociodemographic, clinical, radiological and other therapies. Survival techniques were used to estimate the incidence rate (IR) and 95% CI of functional impairment, expressed per 100 patient-semesters. Cox multivariate regression models were run to examine the influence of RTX and other covariates on FI. Results were expressed as the hazard ratio (HR) and CI. RESULTS:A total of 68 patients were included. FI occurred in 42 patients [IR 23.5 (95% CI 19, 29.1)] and 50% of them had FI within 1.75 years of an ILD diagnosis. A multivariate analysis showed that RTX exposure resulted in a lower risk of FI compared with non-exposure [HR 0.51 (95% CI 0.31, 0.85)]. Interstitial pneumonia, glucocorticoids, disease activity and duration also influenced FI. CONCLUSION:RA-ILD patients deteriorate over time, with the median time free of impairment being <2 years. Patients exposed to RTX had a higher probability of remaining free of FI compared with other therapies. Other factors have also been identified. Key words: rheumatoid arthritis, interstitial lung disease, observational study, rituximab and prognosis

翻译标题与摘要 下载文献
影响因子:4.40
发表时间:2020-01-01
DOI:10.1007/s00262-019-02431-8
作者列表:["Shibaki, Ryota","Murakami, Shuji","Matsumoto, Yuji","Yoshida, Tatsuya","Goto, Yasushi","Kanda, Shintaro","Horinouchi, Hidehito","Fujiwara, Yutaka","Yamamoto, Nobuyuki","Kusumoto, Masahiko","Yamamoto, Noboru","Ohe, Yuichiro"]

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翻译标题与摘要 下载文献
影响因子:4.04
发表时间:2020-01-25
来源期刊:New biotechnology
DOI:10.1016/j.nbt.2019.08.006
作者列表:["Sousa SA","Soares-Castro P","Seixas AMM","Feliciano JR","Balugas B","Barreto C","Pereira L","Santos PM","Leitão JH"]

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