PMID- 34416614
OWN - NLM
STAT- MEDLINE
DCOM- 20220208
LR  - 20220208
IS  - 1532-3064 (Electronic)
IS  - 0954-6111 (Linking)
VI  - 187
DP  - 2021 Oct
TI  - Clinical spectrum of children with interstitial pneumonia with autoimmune 
      features.
PG  - 106566
LID - S0954-6111(21)00272-9 [pii]
LID - 10.1016/j.rmed.2021.106566 [doi]
AB  - 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.
CI  - Copyright (c) 2021 Elsevier Ltd. All rights reserved.
FAU - Ademhan Tural, Dilber
AU  - Ademhan Tural D
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey. Electronic address: 
      dilberademhan@gmail.com.
FAU - Emiralioglu, Nagehan
AU  - Emiralioglu N
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Ozsezen, Beste
AU  - Ozsezen B
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Sunman, Birce
AU  - Sunman B
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Nayir Buyuksahin, Halime
AU  - Nayir Buyuksahin H
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Guzelkas, Ismail
AU  - Guzelkas I
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Oguz, Berna
AU  - Oguz B
AD  - Department of Radiology, School of Medicine, Hacettepe University, Ankara, 
      Turkey.
FAU - Bilginer, Yelda
AU  - Bilginer Y
AD  - Department of Pediatric Rheumatology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Orhan, Diclehan
AU  - Orhan D
AD  - Department of Pediatric Pathology, School of Medicine, Hacettepe University, 
      Ankara, Turkey.
FAU - Yalcin, Ebru
AU  - Yalcin E
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Dogru, Deniz
AU  - Dogru D
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Ozcelik, Ugur
AU  - Ozcelik U
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Ozen, Seza
AU  - Ozen S
AD  - Department of Pediatric Rheumatology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
FAU - Kiper, Nural
AU  - Kiper N
AD  - Department of Pediatric Pulmonology, School of Medicine, Hacettepe University, 
      Ihsan Dogramaci Children's Hospital, Ankara, Turkey.
LA  - eng
PT  - Journal Article
DEP - 20210812
PL  - England
TA  - Respir Med
JT  - Respiratory medicine
JID - 8908438
RN  - 0 (Glucocorticoids)
RN  - 0 (Immunosuppressive Agents)
SB  - IM
MH  - Adolescent
MH  - Age Factors
MH  - Autoimmune Diseases/*complications
MH  - Child
MH  - Female
MH  - Glucocorticoids/therapeutic use
MH  - Humans
MH  - Immunosuppressive Agents/therapeutic use
MH  - Lung Diseases, Interstitial/*diagnosis/drug therapy/etiology/*physiopathology
MH  - Male
MH  - Retrospective Studies
OTO - NOTNLM
OT  - Connective tissue disease (CTD)
OT  - Idiopathic interstitial pneumonia
OT  - Interstitial lung disease (ILD)
OT  - Interstitial pneumonia with autoimmune features (IPAF)
EDAT- 2021/08/21 06:00
MHDA- 2022/02/09 06:00
CRDT- 2021/08/20 20:26
PHST- 2021/01/15 00:00 [received]
PHST- 2021/06/08 00:00 [revised]
PHST- 2021/08/04 00:00 [accepted]
PHST- 2021/08/21 06:00 [pubmed]
PHST- 2022/02/09 06:00 [medline]
PHST- 2021/08/20 20:26 [entrez]
AID - S0954-6111(21)00272-9 [pii]
AID - 10.1016/j.rmed.2021.106566 [doi]
PST - ppublish
SO  - Respir Med. 2021 Oct;187:106566. doi: 10.1016/j.rmed.2021.106566. Epub 2021 Aug 
      12.