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Antibody predictors of mortality and lung function trends in myositis spectrum interstitial lung disease.
Hannah, Jennifer R; Lawrence, Alexandra; Martinovic, Jennifer; Naqvi, Marium; Chua, Felix; Kouranos, Vasileios; Ali, Saadia Sasha; Stock, Carmel; Owens, Cara; Devaraj, Anand; Pollard, Louise; Agarwal, Sangita; Atienza-Mateo, Belén; González-Gay, Miguel Angel; Patel, Amit; West, Alex; Tinsley, Kate; Robbie, Hasti; Lams, Boris; Wells, Athol U; Norton, Sam; Galloway, James; Renzoni, Elisabetta A; Gordon, Patrick A.
Afiliação
  • Hannah JR; Department of Academic Rheumatology, King's College London, London, UK.
  • Lawrence A; Deparment of Rheumatology, King's College Hospital, London, UK.
  • Martinovic J; Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK.
  • Naqvi M; Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK.
  • Chua F; Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK.
  • Kouranos V; Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Ali SS; Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK.
  • Stock C; Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Owens C; Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK.
  • Devaraj A; Department of Academic Rheumatology, King's College London, London, UK.
  • Pollard L; Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK.
  • Agarwal S; Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Atienza-Mateo B; Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK.
  • González-Gay MA; Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Patel A; Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • West A; Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK.
  • Tinsley K; Department of Rheumatology, University Hospital Lewisham, London, UK.
  • Robbie H; Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK.
  • Lams B; Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.
  • Wells AU; Department of Rheumatology, Marques de Valdecilla University Hospital, Santander, Spain.
  • Norton S; Department of Rheumatology, IIS-Fundación Jiménez Díaz, Madrid, Spain.
  • Galloway J; Department of Medicine and Psychiatry, University of Cantabria; Santander, Spain.
  • Renzoni EA; Department of Academic Rheumatology, King's College London, London, UK.
  • Gordon PA; Department of Respiratory Medicine, Guys and St Thomas' NHS Trust, London, UK.
Article em En | MEDLINE | ID: mdl-38039151
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.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Ano de publicação: 2023 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Ano de publicação: 2023 Tipo de documento: Article