Interstitial pneumonia with autoimmune features: challenges and controversies

被引:19
作者
Mackintosh, John A. [1 ]
Wells, Athol U. [2 ,3 ]
Cottin, Vincent [4 ,5 ]
Nicholson, Andrew G. [6 ,7 ]
Renzoni, Elisabetta A. [2 ,3 ]
机构
[1] Prince Charles Hosp, Dept Thorac Med, Brisbane, Qld, Australia
[2] Royal Brompton Hosp, Interstitial Lung Dis Unit, London, England
[3] Imperial Coll, Margaret Turner Warwick Ctr Fibrosing Lung Dis, NHLI, London, England
[4] Hosp Civils Lyon, Louis Pradel Hosp, Natl Reference Ctr Rare Pulm Dis, Lyon, France
[5] Univ Lyon, Claude Bernard Univ Lyon 1, ERN LUNG, INRAE,IVPC,UMR754, Lyon, France
[6] Royal Brompton Hosp, Dept Histopathol, London, England
[7] Imperial Coll, Natl Heart & Lung Inst, London, England
关键词
CONNECTIVE-TISSUE DISEASE; IDIOPATHIC INFLAMMATORY MYOPATHIES; LUNG-DISEASE; CLASSIFICATION CRITERIA; RHEUMATOLOGY/EUROPEAN LEAGUE; AMERICAN-COLLEGE; MYCOPHENOLATE-MOFETIL; SYSTEMIC-SCLEROSIS; FORMES-FRUSTES; DOUBLE-BLIND;
D O I
10.1183/16000617.0177-2021
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
The presence of clinical, serological and/or radiological features suggestive, but not confirmatory, of a defined connective tissue disease in patients with interstitial lung disease is a relatively frequent occurrence. In 2015, the European Respiratory Society and the American Thoracic Society proposed classification criteria for the interstitial pneumonia with autoimmune features (IPAF) research entity to capture such patients in a standardised manner, with the intention of nurturing clinical research. This initiative resulted in the publication of several series of IPAF patients, with significant variation between cohorts in clinical characteristics, outcome and the application of IPAF criteria in patient selection. From this increasing body of published work, it has become apparent that revision of IPAF criteria is now required in order to justify the eventual designation of IPAF as a standalone diagnostic term, as opposed to a provisional entity put forward as a basis for clinical research. This review covers the current state of IPAF, conclusions that can and cannot be drawn from the IPAF evidence base, and ongoing uncertainties that require further expert group consideration.
引用
收藏
页数:19
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