AP-HP, Hôpital Universitaire Necker-Enfants Malades, Service de Pneumologie Pédiatrique, Centre de Référence pour les Maladies Respiratoires Rares de l'Enfant, Paris, France.
Institut Imagine, INSERM UMRS 1163, Paris, France.
Pediatr Pulmonol. 2023 Nov;58(11):3303-3313. doi: 10.1002/ppul.26660. Epub 2023 Sep 13.
Interstitial pneumonia with autoimmune features (IPAF) has been defined for adults with interstitial lung disease (ILD) and autoimmunity who do not meet the criteria for a specific connective tissue disease (CTD). We aimed to determine whether IPAF criteria could apply to children.
We retrospectively studied patients with ILD and autoimmunity followed at Necker Hospital between 2008 and 2019. Children were classified according to specific CTD and IPAF criteria. The epidemiology and course of the disease were studied according to the final diagnosis.
Among 27 patients, 6 fulfilled the criteria for IPAF and represented 4.5% of all patients with ILD during the study period. Other diagnoses included juvenile dermatomyositis (30%), overlap syndromes (19%), systemic lupus erythematosus (15%), systemic sclerosis (7%), mixed CTD (4%), and rheumatoid arthritis (4%). IPAF patients were more frequently boys versus CTD-ILD patients (67% vs. 14%, p = .02). Two patients had severe respiratory distress that led to death for one of them. The course was favorable for the others, with a good response to steroids. The course tended to be more favorable for IPAF patients than for those with CTD-ILD (0% lung fibrosis in the IPAF group vs. 43% in the CTD-ILD group, p = .07).
We confirmed the existence of IPAF in children. Its prevalence was lower than in adults but comparable to that found for other pediatric series. Boys were more highly represented than in CTD-ILD. The course was favorable for most cases. Larger and more prospective studies are needed to confirm these results.
特发性间质性肺炎伴自身免疫特征（IPAF）已被定义为患有间质性肺疾病（ILD）和自身免疫但不符合特定结缔组织病（CTD）标准的成人。我们旨在确定 IPAF 标准是否适用于儿童。
我们回顾性研究了 2008 年至 2019 年期间在 Necker 医院就诊的患有 ILD 和自身免疫的患者。根据特定的 CTD 和 IPAF 标准对儿童进行分类。根据最终诊断研究疾病的流行病学和病程。
在 27 名患者中，有 6 名符合 IPAF 标准，占研究期间所有 ILD 患者的 4.5%。其他诊断包括幼年皮肌炎（30%）、重叠综合征（19%）、系统性红斑狼疮（15%）、系统性硬化症（7%）、混合 CTD（4%）和类风湿关节炎（4%）。IPAF 患者中男孩比 CTD-ILD 患者更常见（67% vs. 14%，p=0.02）。有 2 名患者出现严重呼吸窘迫，其中 1 名死亡。其他患者的病程较好，对类固醇反应良好。与 CTD-ILD 患者相比，IPAF 患者的病程更有利（IPAF 组无肺纤维化 0%，CTD-ILD 组 43%，p=0.07）。
我们证实了儿童 IPAF 的存在。其患病率低于成人，但与其他儿科系列相似。男孩的比例高于 CTD-ILD。大多数情况下病程较好。需要更大和更前瞻性的研究来证实这些结果。
