Home
SIMRI
Gruppi di Studio
News
Formazione & Ricerca
Per la famiglia
Congressi SIMRI
Area soci
All news by interstitial lung diseases
Interstitial pneumonia with autoimmune features (IPAF): also a pediatric reality?
Interstitial pneumonia with autoimmune features (IPAF) is a form of interstitial lung disease (ILD) in which there are signs and symptoms of lung involvement, similar to those of autoimmune diseases, but without completely meeting the diagnostic criteria to identify them as connective tissue disease-associated ILDs (CTD-ILDs). Research on this condition has been predominantly conducted in adults, and there are fewer pediatric data.
Aoust et al., in this paper published in November 2023, questioned the possibility of extending the definition and criteria applied for the adult population by the task force of experts belonging to the European Respiratory Society and the American Thoracic Society in 2015 to pediatric patients as well. These criteria include the combination of clinical, serological and morphological features. They attempted to provide an answer by going to study the prevalence of IPAF among patients referred to a tertiary care center in France (Necker-Enfants Malades Hospital) and their characteristics using the 2015 Task Force classification. The authors retrospectively studied patients with ILD afferent to the center between 01/01/2008 and 31/12/2019. Of the 174 patients analyzed, 21 were found to have CTD-ILDs or 16% and 6 (4.9%) were classified as IPAF. These 6 patients underwent a series of investigations to enable classification: alveolar broncholavage, genetic testing, analysis of lymphocyte count and serum immunoglobulins, ACE assay, echocardiogram, metabolic investigations, liver enzyme assay and liver ultrasound, urinalysis and creatinine assay, assay of plasma interferon levels, autoimmune evaluation, and finally multidisciplinary (dermatologist, rheumatologist, pediatric ophthalmologist) discussion of the case. The comparison between IPAF and CTD-ILD showed that the former was more frequently male, among symptomatology presented more dry cough and chest pain, and had a more favorable course with good response to steroids (0% pulmonary fibrosis in the IPAF group compared with 43% in the CTD-ILD group).
The study also confirms the presence of PAF in pediatric age with a reduced prevalence compared with adults and comparable with other published pediatric studies (between 4.5 and 6.8%), occurs more frequently in males, and has a favorable course in most cases. However, work with larger populations is needed to confirm these findings and especially to better describe this condition in the pediatric setting.
BIBLIOGRAPHY
Aoust L, Berteloot L, Drabent P, Garcelon N, Bodemer C, Molina TJ, Bader-Meunier B, Hadchouel A. Unclassifiable interstitial lung disease and autoimmunity: Towards IPAF in children? Pediatr Pulmonol. 2023 Nov;58(11):3303-3313. doi: 10.1002/ppul.26660. Epub 2023 Sep 13.
