PMID- 32936097
OWN - NLM
STAT- MEDLINE
DCOM- 20210728
LR  - 20210728
IS  - 2042-8189 (Electronic)
IS  - 1478-2715 (Linking)
VI  - 50
IP  - 3
DP  - 2020 Sep
TI  - Interstitial pneumonia with autoimmune features.
PG  - 247-255
LID - 10.4997/JRCPE.2020.307 [doi]
AB  - Interstitial lung disease (ILD) is an umbrella term for lung disease 
      characterised by inflammation and fibrosis of the interstitium. ILD can be 
      idiopathic or secondary to connective tissue disorders, drugs or environmental 
      exposures. Before labelling it as idiopathic we have to rule out secondary 
      causes. ILD is one of the most common extra-articular manifestations of 
      connective tissue diseases (CTDs), causing significant morbidity and mortality. 
      Patients with pre-existing CTD can develop ILD; some patients develop ILD against 
      the background of either one or two clinical features of a CTD or isolated 
      auto-antibody positivity. The current terminology for such an entity is 
      interstitial pneumonia with autoimmune features (IPAF). The current criterion is 
      based on three domains: clinical, serologic and morphologic. To satisfy the IPAF 
      classification criteria, one needs to satisfy the mandatory criterion with one 
      feature from two of the three domains. Classifying patients with this criterion 
      helps in early initiation of immunosuppression and in monitoring them closely for 
      development of features of a well defined CTD. There are a few limitations like 
      the clinical domain being more skewed towards systemic sclerosis and 
      in flammatory myositis, exclusion of antineutrophilic cytoplasmic antibody (ANCA) 
      and cytoplasmic pattern in antinuclear antibody (ANA). There are no clear 
      protocols for treatment of IPAF and most of the data has been extrapolated from 
      the management of systemic sclerosis (SSc) ILD and idiopathic non-specific c 
      interstitial pneumonia (NSIP). Progressive disease in spite of treatment demands 
      stronger immunosuppressive agents. Studies on the role of antifibrotics in IPAF 
      are underway, with few small studies showing positive outcomes. There are 
      con flicting reports on the survival and outcome of the IPAF cohort. Certain 
      studies suggest that they have better survival compared with idiopathic pulmonary 
      fibrosis (IPF) though other studies contradict this statement.
FAU - Santhanam, Sham
AU  - Santhanam S
AD  - Gleneagles Global Health City, Chennai,Tamil Nadu, India, Email: 
      itsdrsham@gmail.com.
FAU - Mohanasundaram, Kavitha
AU  - Mohanasundaram K
AD  - Saveetha Medical College, Chennai, India.
FAU - Krishnan, Shanmuganandan
AU  - Krishnan S
AD  - Sree Balaji Medical College, Chennai, India.
LA  - eng
PT  - Journal Article
PL  - England
TA  - J R Coll Physicians Edinb
JT  - The journal of the Royal College of Physicians of Edinburgh
JID - 101144324
SB  - IM
MH  - *Autoimmune Diseases/complications/diagnosis
MH  - Cohort Studies
MH  - *Connective Tissue Diseases/complications
MH  - Humans
MH  - *Idiopathic Pulmonary Fibrosis
MH  - *Lung Diseases, Interstitial/diagnosis/etiology
MH  - Tomography, X-Ray Computed
OTO - NOTNLM
OT  - CTD-ILD
OT  - HRCT chest
OT  - connective tissue disorder
OT  - idiopathic pulmonary  fi brosis
OT  - interstitial lung disease
OT  - interstitial pneumonia with autoimmune features
COIS- Written informed consent for the paper to be published (including images, case 
      history and data) was obtained from the patient for publication of this paper, 
      including accompanying images.
EDAT- 2020/09/17 06:00
MHDA- 2021/07/29 06:00
CRDT- 2020/09/16 12:14
PHST- 2020/09/16 12:14 [entrez]
PHST- 2020/09/17 06:00 [pubmed]
PHST- 2021/07/29 06:00 [medline]
AID - 10.4997/JRCPE.2020.307 [doi]
PST - ppublish
SO  - J R Coll Physicians Edinb. 2020 Sep;50(3):247-255. doi: 10.4997/JRCPE.2020.307.