PMID- 33873185 OWN - NLM STAT- MEDLINE DCOM- 20220516 LR - 20220516 IS - 1423-0356 (Electronic) IS - 0025-7931 (Linking) VI - 100 IP - 9 DP - 2021 TI - Baseline Characteristics and Survival of an Australian Interstitial Pneumonia with Autoimmune Features Cohort. PG - 853-864 LID - 10.1159/000515396 [doi] AB - BACKGROUND AND OBJECTIVE: The research term "interstitial pneumonia with autoimmune features" (IPAF) encompasses interstitial lung disease (ILD) patients with autoimmune features not meeting diagnostic criteria for a defined connective tissue disease (CTD). It remains unclear if IPAF is a distinct disease entity with implications for management and prognosis. We describe an Australian IPAF population and compare their baseline characteristics and outcomes with distinct cohorts of idiopathic interstitial pneumonia (IIP), CTD-ILD, and unclassifiable ILD. METHODS: Review of 291 consecutive patients attending a specialist ILD clinic was performed. Patients with a diagnosis of IIP, CTD-ILD, and unclassifiable ILD by ILD-multidisciplinary meeting (ILD-MDM) were included. Patients meeting the IPAF criteria were identified. Baseline clinical data, survival, and progression were compared between ILD groups. RESULTS: 226 patients were included, 36 meeting the IPAF criteria. IPAF patients demonstrated a high prevalence of autoantibodies to tRNA synthetase (35.3%), Ro52 (27.8%), and neutrophilic cytoplasmic antigens (ANCA; 20.0%). IPAF and CTD-ILD patients demonstrated similar clinical characteristics (mean age 66.6 and 63.7 years, respectively, female predominant, frequent CTD-manifestations). Lung function did not differ between ILD groups. Disease severity, pulmonary hypertension (PH), and ILD-MDM diagnosis were strong predictors of worse transplant-free survival (TFS). Meeting the IPAF criteria was not associated with TFS. CONCLUSIONS: We identified IPAF as a heterogeneous phenotype that overlaps considerably with CTD-ILD. Disease severity, PH, and ILD-MDM diagnosis were more powerful predictors of survival outcomes than meeting the IPAF criteria. CI - (c) 2021 S. Karger AG, Basel. FAU - Jee, Adelle S AU - Jee AS AD - Department of Respiratory, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. AD - Central Clinical School, University of Sydney, Sydney, New South Wales, Australia. AD - National Health and Medical Research Council (NHMRC) Centre of Research Excellence in Pulmonary Fibrosis, Sydney, New South Wales, Australia. FAU - Parker, Matthew J S AU - Parker MJS AD - Central Clinical School, University of Sydney, Sydney, New South Wales, Australia. AD - National Health and Medical Research Council (NHMRC) Centre of Research Excellence in Pulmonary Fibrosis, Sydney, New South Wales, Australia. AD - Department of Rheumatology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. FAU - Bleasel, Jane F AU - Bleasel JF AD - Central Clinical School, University of Sydney, Sydney, New South Wales, Australia. AD - Department of Rheumatology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. FAU - Troy, Lauren K AU - Troy LK AD - Department of Respiratory, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. AD - Central Clinical School, University of Sydney, Sydney, New South Wales, Australia. FAU - Lau, Edmund M AU - Lau EM AD - Department of Respiratory, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. AD - Central Clinical School, University of Sydney, Sydney, New South Wales, Australia. FAU - Jo, Helen E AU - Jo HE AD - Department of Respiratory, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. AD - Central Clinical School, University of Sydney, Sydney, New South Wales, Australia. AD - National Health and Medical Research Council (NHMRC) Centre of Research Excellence in Pulmonary Fibrosis, Sydney, New South Wales, Australia. FAU - Teoh, Alan K Y AU - Teoh AKY AD - Department of Respiratory, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. AD - Central Clinical School, University of Sydney, Sydney, New South Wales, Australia. AD - National Health and Medical Research Council (NHMRC) Centre of Research Excellence in Pulmonary Fibrosis, Sydney, New South Wales, Australia. FAU - Adelstein, Stephen AU - Adelstein S AD - Central Clinical School, University of Sydney, Sydney, New South Wales, Australia. AD - Central Immunology Laboratory, NSW Health Pathology, Sydney, New South Wales, Australia. AD - Department of Clinical Immunology and Allergy, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. FAU - Webster, Susanne AU - Webster S AD - Department of Respiratory, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. FAU - Corte, Tamera J AU - Corte TJ AD - Department of Respiratory, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. AD - Central Clinical School, University of Sydney, Sydney, New South Wales, Australia. AD - National Health and Medical Research Council (NHMRC) Centre of Research Excellence in Pulmonary Fibrosis, Sydney, New South Wales, Australia. LA - eng PT - Journal Article DEP - 20210419 PL - Switzerland TA - Respiration JT - Respiration; international review of thoracic diseases JID - 0137356 SB - IM MH - Australia/epidemiology MH - *Connective Tissue Diseases/complications MH - Female MH - Humans MH - *Idiopathic Interstitial Pneumonias/diagnosis MH - *Lung Diseases, Interstitial/diagnosis MH - Tomography, X-Ray Computed OTO - NOTNLM OT - Autoimmune diseases OT - Clinical research OT - Connective tissue disease OT - Interstitial lung disease OT - Pulmonary fibrosis associated with collagen vascular disorders EDAT- 2021/04/20 06:00 MHDA- 2022/05/17 06:00 CRDT- 2021/04/19 20:30 PHST- 2020/11/12 00:00 [received] PHST- 2021/02/22 00:00 [accepted] PHST- 2021/04/20 06:00 [pubmed] PHST- 2022/05/17 06:00 [medline] PHST- 2021/04/19 20:30 [entrez] AID - 000515396 [pii] AID - 10.1159/000515396 [doi] PST - ppublish SO - Respiration. 2021;100(9):853-864. doi: 10.1159/000515396. Epub 2021 Apr 19.