PMID- 31494587
OWN - NLM
STAT- MEDLINE
DCOM- 20200210
LR  - 20210907
IS  - 1757-790X (Electronic)
IS  - 1757-790X (Linking)
VI  - 12
IP  - 9
DP  - 2019 Sep 6
TI  - Interstitial pneumonia with autoimmune features and platypnea-orthopnea syndrome.
LID - 10.1136/bcr-2019-230948 [doi]
LID - e230948
AB  - Interstitial pneumonia with autoimmune features (IPAF) is a recently proposed 
      terminology for interstitial lung disease (ILD) with evidence of autoimmunity 
      that does not meet the criteria for a defined connective tissue disease (CTD). 
      Although ILD is well recognised in patients with established CTD, it is rarely 
      the sole presenting feature of CTD. We report a case of 22-year-old male patient, 
      who presented with progressive shortness of breath for 2 months and had features 
      suggestive of platypnea-orthodeoxia syndrome (POS). Imaging revealed ILD with 
      usual interstitial pneumonia pattern. Patient had features of autoimmune disorder 
      but did not fulfil the criteria for any CTD and hence was labelled as IPAF. His 
      POS was attributed predominantly to the lower lobe disease. The patient responded 
      well to immunosuppressive treatment. A systematic review of literature of all 
      cases with POS due to pulmonary parenchymal involvement has also been done.
CI  - (c) BMJ Publishing Group Limited 2019. No commercial re-use. See rights and 
      permissions. Published by BMJ.
FAU - Mathew, Uthara
AU  - Mathew U
AD  - Medicine, All India Institute of Medical Sciences, New Delhi, India.
FAU - Mittal, Ankit
AU  - Mittal A
AD  - Medicine, All India Institute of Medical Sciences, New Delhi, India.
FAU - Vyas, Surabhi
AU  - Vyas S
AD  - Radiology, All India Institute of Medical Sciences, New Delhi, India.
FAU - Ray, Animesh
AU  - Ray A
AUID- ORCID: 0000-0001-7498-9112
AD  - Medicine, All India Institute of Medical Sciences, New Delhi, India.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20190906
PL  - England
TA  - BMJ Case Rep
JT  - BMJ case reports
JID - 101526291
RN  - 0 (Immunosuppressive Agents)
SB  - IM
MH  - Autoimmune Diseases/*diagnosis/drug therapy/immunology/physiopathology
MH  - Dyspnea/immunology
MH  - Humans
MH  - Idiopathic Pulmonary Fibrosis/*diagnosis/drug therapy/immunology/physiopathology
MH  - Immunosuppressive Agents/*therapeutic use
MH  - Male
MH  - Syndrome
MH  - Treatment Outcome
MH  - Young Adult
PMC - PMC6731928
OTO - NOTNLM
OT  - connective tissue disease
OT  - interstitial lung disease
OT  - statistics and research methods
COIS- Competing interests: None declared.
EDAT- 2019/09/09 06:00
MHDA- 2020/02/11 06:00
PMCR- 2021/09/06
CRDT- 2019/09/09 06:00
PHST- 2019/09/09 06:00 [entrez]
PHST- 2019/09/09 06:00 [pubmed]
PHST- 2020/02/11 06:00 [medline]
PHST- 2021/09/06 00:00 [pmc-release]
AID - 12/9/e230948 [pii]
AID - bcr-2019-230948 [pii]
AID - 10.1136/bcr-2019-230948 [doi]
PST - epublish
SO  - BMJ Case Rep. 2019 Sep 6;12(9):e230948. doi: 10.1136/bcr-2019-230948.