Clinical spectrum of children with interstitial pneumonia with autoimmune features - 19/10/21
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Abstract |
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.
Le texte complet de cet article est disponible en PDF.Highlights |
• | Is interstitial pneumonia with autoimmune features (IPAF) seen in children? |
• | Patients with ILD who have findings of autoimmunity without meet the criteria for a certain rheumatic disease are considered to have IPAF. |
• | IPAF is rarely seen in children. |
• | The demographic, clinical, radiologic, serologic and histopathologic features of children with IPAF were defined. |
Keywords : Interstitial lung disease (ILD), Interstitial pneumonia with autoimmune features (IPAF), Connective tissue disease (CTD), Idiopathic interstitial pneumonia
Abbreviations : IPAF, ILD, chILD, CDT, ERS, ATS, HRCT, ANA, NSIP, OP, LIP, UIP
Plan
Vol 187
Article 106566- octobre 2021 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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