Characterisation of patients with interstitial pneumonia with autoimmune features.
Characterisation of patients with interstitial pneumonia with autoimmune features.
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DOI：
10.1183/13993003.01565-2015
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发表时间：
2016-06
期刊：
影响因子：
--
通讯作者：
Strek ME
中科院分区：
文献类型：
--
作者：
Oldham JM;Adegunsoye A;Valenzi E;Lee C;Witt L;Chen L;Husain AN;Montner S;Chung JH;Cottin V;Fischer A;Noth I;Vij R;Strek ME
间质性肺病（ILD）患者可能具有结缔组织病（CTD）的特征，但缺乏特定CTD的诊断结果。最近欧洲呼吸学会/美国胸科学会研究声明提出了具有自身免疫特征的间质性肺炎（IPAF）患者的标准。我们将IPAF标准应用于特发性间质性肺炎和未分化CTD-ILD（UCTD）患者。然后，我们描述了IPAF队列的临床、血清学和形态学特征，将结局与其他ILD队列进行了比较，并以生存期为终点验证了单个IPAF领域。在422例患者中，144例符合IPAF标准。平均年龄为63.2岁，女性略多。IPAF队列生存率略高于特发性肺纤维化患者，但低于CTD-ILD。一个不常见的间质性肺炎模式与生存率的提高，因为是临床领域的存在。符合临床领域和形态学领域内放射学或组织学特征的改良IPAF队列显示出与CTD-ILD患者相似的生存率。IPAF在特发性间质性肺炎和UCTD患者中很常见。特定的IPAF功能可以识别具有不同生存率的亚组。需要进一步的研究来复制这些发现，并确定符合IPAF标准的患者是否从免疫抑制治疗中获益。
Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF). We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint. Of 422 patients, 144 met IPAF criteria. Mean age was 63.2 years with a slight female predominance. IPAF cohort survival was marginally better than patients with idiopathic pulmonary fibrosis, but worse than CTD-ILD. A non-usual interstitial pneumonia pattern was associated with improved survival, as was presence of the clinical domain. A modified IPAF cohort of those meeting the clinical domain and a radiographic or histological feature within the morphological domain displayed survival similar to those with CTD-ILD. IPAF is common among patients with idiopathic interstitial pneumonia and UCTD. Specific IPAF features can identify subgroups with differential survival. Further research is needed to replicate these findings and determine whether patients meeting IPAF criteria benefit from immunosuppressive therapy.
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