An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features
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DOI:
10.1183/13993003.00150-2015
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发表时间:
2015-10-01
影响因子:
24.3
通讯作者:
Cottin, Vincent
Cottin, Vincent
中科院分区:
医学1区
文献类型:
--
作者:
Fischer, Aryeh;Antoniou, Katerina M.;Cottin, Vincent

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许多特发性间质性肺炎(IIP)患者的临床特征提示潜在的自身免疫过程,但不符合结缔组织病(CTD)的既定标准。研究人员提出了不同的标准和术语来描述这些患者,在命名和分类上缺乏共识,限制了对统一队列进行前瞻性研究的能力。“欧洲呼吸学会/美国胸科学会未分化结缔组织病相关间质性肺病工作组”是为了就HP患者的命名和分类标准以及自身免疫的特征达成共识而成立的。该工作组提出了术语“具有自身免疫特征的间质性肺炎”(IPAF)并提供了围绕来自三个领域的特征组合的存在而组织的分类标准:由特异性胸外特征组成的临床领域,由特异性自身抗体组成的血清学领域,以及由特异性胸部成像、组织病理学或肺生理学特征组成的形态学领域。IPAF的名称应用于识别TIP患者和提示以下特征的特征,但还不能确定是CTD IPAF提供了一个良好的平台,可以从这个平台上启动对更统一的队列的必要的未来研究调查。
Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). Researchers have proposed differing criteria and terms to describe these patients, and lack of consensus over nomenclature and classification limits the ability to conduct prospective studies of a uniform cohort.The "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" was formed to create consensus regarding the nomenclature and classification criteria for patients with HP and features of autoimmunity.The task force proposes the term "interstitial pneumonia with autoimmune features" (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantiboclies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features.A designation of IPAF should be used to identify individuals with TIP and features suggestive of, but not definitive for, a CTD. With IPAF, a sound platform has been provided from which to launch the requisite future research investigations of a more uniform cohort.