Background

Interstitial pneumonia with autoimmune features (IPAF) is a term used to describe adult patients with interstitial lung disease (ILD) who display some clinical or serological features of autoimmune diseases and who do not meet the full criteria for a specific connective tissue disease (CTD). The aim of this study was to define the demographic, clinical, radiologic, serologic and histopathologic features and assess treatment in children with IPAF.

Methods

This retrospective cohort study was conducted at a tertiary referral pediatric pulmonology center between January 2010 and August 2020. Children with proven interstitial pneumonia with no known etiologies were evaluated for IPAF according to European Respiratory Society/American Thoracic Society research statement of IPAF.

Results

Among 132 children with ILD, 17 patients were evaluated in detail for IPAF criteria and six patients were further diagnosed as having IPAF. The incidence of IPAF in our patients with ILD was 4.5%. Four of these patients were female. The median age at the time of ILD diagnosis was 10.5 years. The most common initial symptom was shortness of breath, and the most common physical examination sign was crackles in both lungs. Steroid therapy was given to all patients and four patients received other immunosuppressive agents for steroid sparing. Two of those patients died because of respiratory insufficiency during the follow-up.

Conclusion

Children with interstitial pneumonia and certain clinical, serologic, and/or morphologic features should raise suspicion for the presence of an underlying systemic autoimmune disease. IPAF is also seen in children and should be categorized in chILD classifications.

中文翻译：

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具有自身免疫特征的间质性肺炎患儿的临床表现

背景

具有自身免疫特征的间质性肺炎 (IPAF) 是一个术语，用于描述具有自身免疫性疾病的某些临床或血清学特征且不符合特定结缔组织病 (CTD) 的完整标准的间质性肺病 (ILD) 成年患者. 本研究的目的是确定 IPAF 儿童的人口统计学、临床、放射学、血清学和组织病理学特征并评估治疗。

方法

这项回顾性队列研究于 2010 年 1 月至 2020 年 8 月在一家三级转诊儿科肺病学中心进行。根据欧洲呼吸学会/美国胸科学会 IPAF 研究声明，对确诊为无已知病因的间质性肺炎的儿童进行了 IPAF 评估。

结果

在 132 名 ILD 儿童中，17 名患者接受了 IPAF 标准的详细评估，6 名患者进一步诊断为 IPAF。我们的 ILD 患者 IPAF 的发生率为 4.5%。其中四名患者是女性。ILD 诊断时的中位年龄为 10.5 岁。最常见的初始症状是气短，最常见的体格检查体征是双肺湿啰音。所有患者均接受了类固醇治疗，4 名患者接受了其他免疫抑制剂以节省类固醇。其中两名患者在随访期间因呼吸功能不全而死亡。

结论

患有间质性肺炎和某些临床、血清学和/或形态学特征的儿童应怀疑是否存在潜在的全身性自身免疫性疾病。IPAF 也见于儿童，应归入儿童分类。