Clinical spectrum of children with interstitial pneumonia with autoimmune features:a new definition in childhood interstitial lung diseases

Abstract

<b>Background:</b> Interstitial Pneumonia with Autoimmune Features (IPAF) describes in adult patients with interstitial lung disease (ILD) based on clinical or serological features of autoimmune diseases insufficient to diagnose 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. <b>Methods:</b> This retrospective cohort study was conducted at a tertiary referral pediatric pulmonology center between January 2010 to August 2020. Children with proven interstitial pneumonia excluding other etiologies for interstitial pneumonia were evaluated for IPAF according to ERSociety/ATS research statement of IPAF. <b>Results:</b> Among 132 children with ILD, 1 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% (6/132). 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 of 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 median follow-up of 5.5 years. <b>Conclusion:</b> 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.