Fernandes Ana Luísa, Ferreira Jorge, Neves Inês
Pulmonology Department, Hospital Pedro Hispano, Matosinhos, Portugal.
Respir Med Case Rep. 2021 Jan 20;32:101350. doi: 10.1016/j.rmcr.2021.101350. eCollection 2021.
The topic of interstitial pneumonia with autoimmune features (IPAF) is a research classification proposed by the European Respiratory Society/American Thoracic Society Task Force: this includes patients with idiopathic interstitial pneumonia (IIP) and clinical features, suggesting an underlying autoimmune process, but who do not meet established criteria for a connective tissue disease (CTD). We aimed to perform a detailed characterization of clinical, serological, and radiological features for our patients with IPAF criteria. Six patients were included, and a comprehensive description of these cases revealed a heterogeneous group in terms of clinical and treatment options. In most patients, it was possible to identify other features and disorders with an autoimmune "background," which may support the inclusion of these patients in the IPAF classification. No deaths or significant decline in lung function occurred, and thus no definitive diagnosis of CTD could be found over 35 months of median follow-up. Therefore, IPAF is a recent concept, with many questions still open in regard to its usage in the ILD field.
具有自身免疫特征的间质性肺炎（IPAF）这一主题是欧洲呼吸学会/美国胸科学会特别工作组提出的一种研究分类：这包括患有特发性间质性肺炎（IIP）且具有临床特征，提示存在潜在自身免疫过程，但不符合结缔组织病（CTD）既定标准的患者。我们旨在对符合IPAF标准的患者的临床、血清学和放射学特征进行详细描述。纳入了6例患者，对这些病例的全面描述显示，在临床和治疗选择方面，这是一组异质性患者。在大多数患者中，有可能识别出具有自身免疫“背景”的其他特征和疾病，这可能支持将这些患者纳入IPAF分类。未发生死亡或肺功能显著下降，因此在中位随访35个月期间未发现明确的CTD诊断。因此，IPAF是一个较新的概念，在ILD领域的应用方面仍有许多问题有待解决。
