PMID- 31709050 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200929 IS - 2050-0068 (Print) IS - 2050-0068 (Electronic) IS - 2050-0068 (Linking) VI - 8 IP - 11 DP - 2019 TI - Idiopathic and immune-related pulmonary fibrosis: diagnostic and therapeutic challenges. PG - e1086 LID - 10.1002/cti2.1086 [doi] LID - e1086 AB - Interstitial lung disease (ILD) encompasses a large group of pulmonary conditions sharing common clinical, radiological and histopathological features as a consequence of fibrosis of the lung interstitium. The majority of ILDs are idiopathic in nature with possible genetic predisposition, but is also well recognised as a complication of connective tissue disease or with certain environmental, occupational or drug exposures. In recent years, a concerted international effort has been made to standardise the diagnostic criteria in ILD subtypes, formalise multidisciplinary pathways and standardise treatment recommendations. In this review, we discuss some of the current challenges around ILD diagnostics, the role of serological testing, especially, in light of the new classification of Interstitial Pneumonia with Autoimmune Features (IPAF) and discuss the evidence for therapies targeted at idiopathic and immune-related pulmonary fibrosis. CI - (c) 2019 The Authors. Clinical & Translational Immunology published by John Wiley & Sons Australia, Ltd on behalf of Australian and New Zealand Society for Immunology Inc. FAU - McLean-Tooke, Andrew AU - McLean-Tooke A AUID- ORCID: 0000-0003-4307-0837 AD - Department of Clinical Immunology Sir Charles Gairdner Hospital Perth WA Australia. AD - Department of Laboratory Immunology PathWest QEII Medical Centre Perth WA Australia. FAU - Moore, Irene AU - Moore I AD - Department of Respiratory Medicine Fiona Stanley Hospital Perth WA Australia. FAU - Lake, Fiona AU - Lake F AD - Department of Respiratory Medicine Sir Charles Gairdner Hospital Perth WA Australia. LA - eng PT - Journal Article PT - Review DEP - 20191105 PL - Australia TA - Clin Transl Immunology JT - Clinical & translational immunology JID - 101638268 PMC - PMC6831929 OTO - NOTNLM OT - connective tissue disease OT - diagnosis OT - interstitial lung diseases (ILDs) OT - pulmonary fibrosis OT - therapeutics COIS- IM has received funding to attend an educational meeting from Boerigher Ingelheim, the manufacturers of nintenadib. AMT and FL declare no conflicts of interest. EDAT- 2019/11/12 06:00 MHDA- 2019/11/12 06:01 PMCR- 2019/11/05 CRDT- 2019/11/12 06:00 PHST- 2019/04/17 00:00 [received] PHST- 2019/09/29 00:00 [revised] PHST- 2019/10/10 00:00 [accepted] PHST- 2019/11/12 06:00 [entrez] PHST- 2019/11/12 06:00 [pubmed] PHST- 2019/11/12 06:01 [medline] PHST- 2019/11/05 00:00 [pmc-release] AID - CTI21086 [pii] AID - 10.1002/cti2.1086 [doi] PST - epublish SO - Clin Transl Immunology. 2019 Nov 5;8(11):e1086. doi: 10.1002/cti2.1086. eCollection 2019.