Department of Respiratory, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
Central Clinical School, University of Sydney, Sydney, New South Wales, Australia.
Respiration. 2021;100(9):853-864. doi: 10.1159/000515396. Epub 2021 Apr 19.
The research term "interstitial pneumonia with autoimmune features" (IPAF) encompasses interstitial lung disease (ILD) patients with autoimmune features not meeting diagnostic criteria for a defined connective tissue disease (CTD). It remains unclear if IPAF is a distinct disease entity with implications for management and prognosis. We describe an Australian IPAF population and compare their baseline characteristics and outcomes with distinct cohorts of idiopathic interstitial pneumonia (IIP), CTD-ILD, and unclassifiable ILD.
Review of 291 consecutive patients attending a specialist ILD clinic was performed. Patients with a diagnosis of IIP, CTD-ILD, and unclassifiable ILD by ILD-multidisciplinary meeting (ILD-MDM) were included. Patients meeting the IPAF criteria were identified. Baseline clinical data, survival, and progression were compared between ILD groups.
226 patients were included, 36 meeting the IPAF criteria. IPAF patients demonstrated a high prevalence of autoantibodies to tRNA synthetase (35.3%), Ro52 (27.8%), and neutrophilic cytoplasmic antigens (ANCA; 20.0%). IPAF and CTD-ILD patients demonstrated similar clinical characteristics (mean age 66.6 and 63.7 years, respectively, female predominant, frequent CTD-manifestations). Lung function did not differ between ILD groups. Disease severity, pulmonary hypertension (PH), and ILD-MDM diagnosis were strong predictors of worse transplant-free survival (TFS). Meeting the IPAF criteria was not associated with TFS.
We identified IPAF as a heterogeneous phenotype that overlaps considerably with CTD-ILD. Disease severity, PH, and ILD-MDM diagnosis were more powerful predictors of survival outcomes than meeting the IPAF criteria.
“具有自身免疫特征的间质性肺炎”（IPAF）这一研究术语涵盖了不符合明确结缔组织病（CTD）诊断标准的具有自身免疫特征的间质性肺疾病（ILD）患者。目前尚不清楚 IPAF 是否为一种具有管理和预后意义的独特疾病实体。我们描述了一个澳大利亚 IPAF 人群，并将其基线特征和结局与特发性间质性肺炎（IIP）、CTD-ILD 和无法分类的 ILD 的明确队列进行了比较。
对参加ILD 专家诊所的 291 例连续患者进行了回顾性研究。纳入ILD 多学科会议（ILD-MDM）诊断为 IIP、CTD-ILD 和无法分类的 ILD 的患者。确定符合 IPAF 标准的患者。比较ILD 组之间的基线临床数据、生存和进展情况。
共纳入 226 例患者，其中 36 例符合 IPAF 标准。IPAF 患者表现出 tRNA 合成酶（35.3%）、Ro52（27.8%）和中性粒细胞胞质抗原（ANCA；20.0%）自身抗体的高患病率。IPAF 和 CTD-ILD 患者具有相似的临床特征（分别为 66.6 岁和 63.7 岁的平均年龄，女性为主，常见 CTD 表现）。ILD 组之间的肺功能无差异。疾病严重程度、肺动脉高压（PH）和ILD-MDM 诊断是移植后无进展生存率（TFS）更差的有力预测因素。符合 IPAF 标准与 TFS 无关。
我们发现 IPAF 是一种具有异质性表型的疾病，与 CTD-ILD 重叠程度很大。疾病严重程度、PH 和 ILD-MDM 诊断比符合 IPAF 标准更能预测生存结局。
