PMID- 37701997
OWN - NLM
STAT- MEDLINE
DCOM- 20231023
LR  - 20231024
IS  - 1099-0496 (Electronic)
IS  - 1099-0496 (Linking)
VI  - 58
IP  - 11
DP  - 2023 Nov
TI  - Unclassifiable interstitial lung disease and autoimmunity: Towards IPAF in 
      children?
PG  - 3303-3313
LID - 10.1002/ppul.26660 [doi]
AB  - INTRODUCTION: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.
CI  - (c) 2023 Wiley Periodicals LLC.
FAU - Aoust, Laura
AU  - Aoust L
AD  - AP-HP, Hopital Universitaire Necker-Enfants Malades, Service de Pneumologie 
      Pediatrique, Centre de Reference pour les Maladies Respiratoires Rares de 
      l'Enfant, Paris, France.
FAU - Berteloot, Laureline
AU  - Berteloot L
AD  - Institut Imagine, INSERM UMRS 1163, Paris, France.
AD  - AP-HP, Service d'Imagerie Pediatrique, Hopital Universitaire Necker-Enfants 
      Malades, Paris, France.
FAU - Drabent, Philippe
AU  - Drabent P
AD  - AP-HP, Hopitaux Universitaires Necker-Enfants Malades et Robert Debre, Service 
      d'Anatomie Pathologique, Paris, France.
FAU - Garcelon, Nicolas
AU  - Garcelon N
AD  - Imagine Institute, Data Science Platform, INSERM UMR 1163, Universite de Paris, 
      Paris, France.
FAU - Bodemer, Christine
AU  - Bodemer C
AD  - APHP, Hopital Universitaire Necker-Enfants Malades, Service de Dermatologie 
      Pediatrique, Paris, France.
AD  - Universite Paris Cite, Paris, France.
FAU - Molina, Thierry Jo
AU  - Molina TJ
AD  - Institut Imagine, INSERM UMRS 1163, Paris, France.
AD  - AP-HP, Hopitaux Universitaires Necker-Enfants Malades et Robert Debre, Service 
      d'Anatomie Pathologique, Paris, France.
AD  - Universite Paris Cite, Paris, France.
FAU - Bader-Meunier, Brigitte
AU  - Bader-Meunier B
AD  - APHP, Hopital Universitaire Necker-Enfants Malades, Service d'Immunologie et 
      Rhumatologie Pediatriques, Paris, France.
AD  - Center for Rheumatic, AutoImmune and Systemic Diseases in Children (RAISE), Paris 
      Cite University, Paris, France.
AD  - Laboratory of Immunogenetics of Paediatric Autoimmunity, Imagine Institute, 
      INSERM U1163, Paris Cite University, Paris, France.
FAU - Hadchouel, Alice
AU  - Hadchouel A
AUID- ORCID: 0000-0001-7451-9890
AD  - AP-HP, Hopital Universitaire Necker-Enfants Malades, Service de Pneumologie 
      Pediatrique, Centre de Reference pour les Maladies Respiratoires Rares de 
      l'Enfant, Paris, France.
AD  - Universite Paris Cite, Paris, France.
AD  - INSERM U1151, Institut Necker Enfants Malades, Paris, France.
LA  - eng
PT  - Journal Article
DEP - 20230913
PL  - United States
TA  - Pediatr Pulmonol
JT  - Pediatric pulmonology
JID - 8510590
SB  - IM
MH  - Male
MH  - Humans
MH  - Child
MH  - Autoimmunity
MH  - Retrospective Studies
MH  - *Lung Diseases, Interstitial/epidemiology/diagnosis
MH  - *Connective Tissue Diseases/complications/epidemiology/diagnosis
MH  - *Autoimmune Diseases/complications/epidemiology
OTO - NOTNLM
OT  - autoimmunity
OT  - children
OT  - connective tissue disease
OT  - intersitial lung disease
EDAT- 2023/09/13 06:41
MHDA- 2023/10/23 00:42
CRDT- 2023/09/13 04:23
PHST- 2023/07/14 00:00 [revised]
PHST- 2023/01/04 00:00 [received]
PHST- 2023/08/19 00:00 [accepted]
PHST- 2023/10/23 00:42 [medline]
PHST- 2023/09/13 06:41 [pubmed]
PHST- 2023/09/13 04:23 [entrez]
AID - 10.1002/ppul.26660 [doi]
PST - ppublish
SO  - Pediatr Pulmonol. 2023 Nov;58(11):3303-3313. doi: 10.1002/ppul.26660. Epub 2023 
      Sep 13.