PMID- 38039151 OWN - NLM STAT- Publisher LR - 20231201 IS - 1462-0332 (Electronic) IS - 1462-0324 (Linking) DP - 2023 Dec 1 TI - Antibody predictors of mortality and lung function trends in myositis spectrum interstitial lung disease. LID - kead638 [pii] LID - 10.1093/rheumatology/kead638 [doi] AB - OBJECTIVES: The impact of autoantibody profiles on prognosis of idiopathic inflammatory myositis associated interstitial lung disease (IIM-ILD) and myositis spectrum ILD with Myositis Specific Antibodies (MSA) remains unclear. This retrospective cohort study examines whether serological profiles are associated with mortality and longitudinal lung function change. METHODS: Baseline clinical/demographic characteristics and follow-up lung function of consecutive adult patients with IIM-ILD or Interstitial Pneumonia with Autoimmune Features (IPAF) positive for MSAs were extracted from three hospitals. Univariate and multi-variate Cox-Proportional Hazards analyses were used to compare mortality between autoantibodies. Regression models were used to analyse lung function trends. RESULTS: Of 430 included patients, 81% met IIM criteria, 19% were IPAF-MSA. On univariate analysis, risk factors associated with mortality included higher age, Charlson Co-morbidity Index and CRP; and lower BMI, baseline TLCO% and FEV1%. Compared to anti-MDA5-negativity, anti-MDA5-positivity (MDA5+) was associated with high mortality in the first 3 months (HR 65.2. 95%CI 14.1, 302.0), while no significant difference was seen thereafter (HR 0.55, 95%CI 0.14, 2.28). On multi-variate analysis, combined anti-synthetase antibodies carried a reduced risk of mortality (HR 0.63), although individually, mortality was reduced in anti-Jo1 + (HR 0.61, 95%CI 0.4-0.87) and increased in anti-PL7+ patients (HR 2.07, 95%CI 1.44-2.99). Anti-MDA5+ was associated with slow improvement in %FVC over the first 3 years, while anti-PL7+ was linked with a slow decline from 12 months onwards. CONCLUSIONS: Among autoantibody profiles in myositis spectrum disorders, anti-MDA5+ and anti-PL7+ confer higher mortality risks. Survivors of an early peak of mortality in anti-MDA5+ disease appear to have a favourable prognosis. CI - (c) The Author(s) 2023. Published by Oxford University Press on behalf of the British Society for Rheumatology. FAU - Hannah, Jennifer R AU - Hannah JR AD - Department of Academic Rheumatology, King's College London, London, UK. AD - Deparment of Rheumatology, King's College Hospital, London, UK. FAU - Lawrence, Alexandra AU - Lawrence A AD - Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK. FAU - Martinovic, Jennifer AU - Martinovic J AD - Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK. FAU - Naqvi, Marium AU - Naqvi M AD - Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK. FAU - Chua, Felix AU - Chua F AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Kouranos, Vasileios AU - Kouranos V AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Ali, Saadia Sasha AU - Ali SS AD - Department of Academic Rheumatology, King's College London, London, UK. AD - Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK. FAU - Stock, Carmel AU - Stock C AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Owens, Cara AU - Owens C AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK. FAU - Devaraj, Anand AU - Devaraj A AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK. FAU - Pollard, Louise AU - Pollard L AD - Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK. AD - Department of Rheumatology, University Hospital Lewisham, London, UK. FAU - Agarwal, Sangita AU - Agarwal S AD - Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK. FAU - Atienza-Mateo, Belen AU - Atienza-Mateo B AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Department of Rheumatology, Marques de Valdecilla University Hospital, Santander, Spain. FAU - Gonzalez-Gay, Miguel Angel AU - Gonzalez-Gay MA AD - Department of Rheumatology, IIS-Fundacion Jimenez Diaz, Madrid, Spain. AD - Department of Medicine and Psychiatry, University of Cantabria; Santander, Spain. FAU - Patel, Amit AU - Patel A AD - Department of Academic Rheumatology, King's College London, London, UK. FAU - West, Alex AU - West A AD - Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK. FAU - Tinsley, Kate AU - Tinsley K AD - Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK. FAU - Robbie, Hasti AU - Robbie H AD - Department of Academic Rheumatology, King's College London, London, UK. FAU - Lams, Boris AU - Lams B AD - Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK. FAU - Wells, Athol U AU - Wells AU AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Norton, Sam AU - Norton S AD - Deparment of Rheumatology, King's College Hospital, London, UK. FAU - Galloway, James AU - Galloway J AD - Department of Academic Rheumatology, King's College London, London, UK. AD - Deparment of Rheumatology, King's College Hospital, London, UK. FAU - Renzoni, Elisabetta A AU - Renzoni EA AD - Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK. AD - Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK. FAU - Gordon, Patrick A AU - Gordon PA AD - Department of Academic Rheumatology, King's College London, London, UK. AD - Deparment of Rheumatology, King's College Hospital, London, UK. LA - eng PT - Journal Article DEP - 20231201 PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM OTO - NOTNLM OT - autoantibodies OT - idiopathic inflammatory myopathies OT - interstitial lung disease OT - mortality OT - myositis OT - progression EDAT- 2023/12/01 18:42 MHDA- 2023/12/01 18:42 CRDT- 2023/12/01 12:43 PHST- 2023/09/14 00:00 [received] PHST- 2023/11/06 00:00 [revised] PHST- 2023/11/09 00:00 [accepted] PHST- 2023/12/01 18:42 [medline] PHST- 2023/12/01 18:42 [pubmed] PHST- 2023/12/01 12:43 [entrez] AID - 7457503 [pii] AID - 10.1093/rheumatology/kead638 [doi] PST - aheadofprint SO - Rheumatology (Oxford). 2023 Dec 1:kead638. doi: 10.1093/rheumatology/kead638.