PMID- 38433068
OWN - NLM
STAT- Publisher
LR  - 20240303
IS  - 1768-3122 (Electronic)
IS  - 0248-8663 (Linking)
DP  - 2024 Mar 2
TI  - [The concept of interstitial pneumonia with autoimmune features (IPAF)].
LID - S0248-8663(24)00059-6 [pii]
LID - 10.1016/j.revmed.2024.02.005 [doi]
AB  - Interstitial lung diseases (ILD) are a heterogeneous group of respiratory 
      diseases often related to connective tissue diseases. Some patients will develop 
      an ILD with autoimmune features without reaching the recommended criteria for 
      autoimmune diseases. Their management is difficult because they have both 
      features for idiopathic and connective tissue disease. To better identify these 
      patients, the concept of interstitial pneumonia with autoimmune features (IPAF) 
      has been created. The diagnosis relies on ILD without identified cause and the 
      presence of at least one defined criterion among 2 of the 3 following domains: 
      clinic, serologic, and morphologic. The mean age at diagnosis is 60, a sex ratio 
      of 1/1, and depending on the authors close to 20% of patients with IPAF will 
      develop a connective tissue disease according to the international criterion. 
      Their prognosis is better than for patients with idiopathic ILD and with an 
      average 5-year survival of 70%. Older age at diagnosis, a pattern of usual 
      interstitial pneumonia, and an impaired diffusing capacity for carbon monoxide 
      have been identified as poor prognosis factors. The treatment relies on usual 
      care for chronic respiratory diseases and is often associated with 
      immunosuppressive and/or antifibrotic therapies. The objective of this 
      classification is to better characterize these patients and improve their 
      management.
CI  - Copyright (c) 2024 The Authors. Published by Elsevier Masson SAS.. All rights 
      reserved.
FAU - Bermudez, J
AU  - Bermudez J
AD  - Service de pneumologie, equipe de transplantation pulmonaire adulte, centre de 
      competences nationales des maladies pulmonaires rares, centre de competences 
      nationales de l'hypertension pulmonaire, CRCM adulte, hopital Nord, CHU Nord, 
      Assistance publique-Hopitaux de Marseille, 15, chemin des Bourrely, 13015 
      Marseille, France; Inserm, centre de recherche en cardiovasculaire et nutrition 
      (C2VN), Aix-Marseille universite, Marseille, France. Electronic address: 
      julien.bermudez@ap-hm.fr.
FAU - Habert, P
AU  - Habert P
AD  - Service de radiologie cardiothoracique diagnostique et interventionnelle, hopital 
      Nord, Aix-Marseille universite, AP-HM, Marseille, France; LIIE, Aix-Marseille 
      universite, Marseille, France.
FAU - Coiffard, B
AU  - Coiffard B
AD  - Service de pneumologie, equipe de transplantation pulmonaire adulte, centre de 
      competences nationales des maladies pulmonaires rares, centre de competences 
      nationales de l'hypertension pulmonaire, CRCM adulte, hopital Nord, CHU Nord, 
      Assistance publique-Hopitaux de Marseille, 15, chemin des Bourrely, 13015 
      Marseille, France; Inserm, centre de recherche en cardiovasculaire et nutrition 
      (C2VN), Aix-Marseille universite, Marseille, France.
LA  - fre
PT  - English Abstract
PT  - Journal Article
PT  - Review
TT  - Le concept de pneumopathie interstitielle diffuse avec manifestations 
      auto-immunes (IPAF).
DEP - 20240302
PL  - France
TA  - Rev Med Interne
JT  - La Revue de medecine interne
JID - 8101383
SB  - IM
OTO - NOTNLM
OT  - Auto-immunite
OT  - Autoimmunity
OT  - Connective tissue diseases
OT  - Connectivites indifferenciees
OT  - Fibrose pulmonaire
OT  - Interstitial lung diseases
OT  - Tomodensitometrie
OT  - X-ray computed tomography
EDAT- 2024/03/04 00:43
MHDA- 2024/03/04 00:43
CRDT- 2024/03/03 21:54
PHST- 2023/10/11 00:00 [received]
PHST- 2024/01/02 00:00 [revised]
PHST- 2024/02/11 00:00 [accepted]
PHST- 2024/03/04 00:43 [medline]
PHST- 2024/03/04 00:43 [pubmed]
PHST- 2024/03/03 21:54 [entrez]
AID - S0248-8663(24)00059-6 [pii]
AID - 10.1016/j.revmed.2024.02.005 [doi]
PST - aheadofprint
SO  - Rev Med Interne. 2024 Mar 2:S0248-8663(24)00059-6. doi: 
      10.1016/j.revmed.2024.02.005.