title: Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group.
abstract: To evaluate the clinical manifestations, long-term clinical outcome and longitudinal pulmonary function in a large cohort of Spanish patients with anti-Jo1 antibodies.
  
  
  
  We retrospectively analyzed the clinical data and lung function parameters of 148 anti-Jo1 patients recruited from a multicentre registry including 18 Spanish hospitals. A composite endpoint was defined, comprising death due to respiratory failure directly related to antisynthetase syndrome (ASS), the need for long-term oxygen therapy or lung transplantation.
  
  
  
  Median follow-up was 78.3 months. Clinical presentation patterns at onset are as follows: isolated interstitial lung disease (ILD) (32.4%), isolated myositis (26.9%), concomitant myositis and ILD (22.8%), and isolated polyarthritis (17.9%). Myositis with ILD was the most frequent final clinical phenotype (67.6%). In most ASS patients, ILD was a non-progressive disease, tending to stabilize with therapy. The endpoint was reached in a significantly larger number of ILD patients with dyspnea at onset than those with paucisymptomatic or asymptomatic forms (p = 0.01). A steady FVC decrease was the hallmark of patients with end-stage lung disease. Estimated survival rates were 87.7% and 75.4% at 5 and 10 years, respectively. Cancer (p = 0.02) and advanced age at ASS diagnosis (p < 0.0001) were related to poorer survival. Mortality was significantly higher than in the general Spanish population, with a standardised mortality ratio (95% CI) of 4.03 (2.79-5.64).
  
  
  
  Anti-Jo1 ASS is a heterogeneous syndrome. ILD in ASS under immunosuppressive therapy is mainly a non-progressive disease. Dyspnea at ILD onset and a steady FVC decrease over time were related to a poorer respiratory prognosis.
authors.affiliation: Internal Medicine Department, Vall d'Hebron General Hospital, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain. Electronic address: etrallero@vhebron.net.
authors.forename: Ernesto
authors.initials: E
authors.lastname: Trallero-Araguás
authors.affiliation: Internal Medicine Department, Hospital Clinic, Barcelona, Spain.
authors.forename: Josep María
authors.initials: JM
authors.lastname: Grau-Junyent
authors.affiliation: Internal Medicine Department, Vall d'Hebron General Hospital, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
authors.forename: Anne
authors.initials: A
authors.lastname: Labirua-Iturburu
authors.affiliation: Internal Medicine Department, Hospital Virgen del Rocío, Sevilla, Spain.
authors.forename: Francisco José
authors.initials: FJ
authors.lastname: García-Hernández
authors.affiliation: Internal Medicine Department, Hospital Parc Taulí, Sabadell, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Monteagudo-Jiménez
authors.affiliation: Internal Medicine Department, Hospital Ramón y Cajal, Madrid, Spain.
authors.forename: Guadalupe
authors.initials: G
authors.lastname: Fraile-Rodriguez
authors.affiliation: Internal Medicine Department, Hospital de Cruces, Barakaldo, Spain.
authors.forename: Iñigo
authors.initials: I
authors.lastname: Les-Bujanda
authors.affiliation: Internal Medicine Department, Hospital Universitario Mutua de Terrasa, Terrasa, Spain.
authors.forename: Mónica
authors.initials: M
authors.lastname: Rodriguez-Carballeira
authors.affiliation: Internal Medicine Department, Hospital Miguel Servet, Zaragoza, Spain.
authors.forename: Luis
authors.initials: L
authors.lastname: Sáez-Comet
authors.affiliation: Internal Medicine Department, Vall d'Hebron General Hospital, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
authors.forename: Albert
authors.initials: A
authors.lastname: Selva-O'Callaghan
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000276:immunology*; D001168:Arthritis / Q000150:complications* / Q000276:immunology; D005260:Female; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000150:complications* / Q000276:immunology; D012042:Registries; D012189:Retrospective Studies
chemical_list: D000974:Antibodies, Antinuclear; C046750:Jo-1 antibody
keywords: Anti-Jo1 antibodies; Antisynthetase syndrome; Idiopathic Inflammatory myopathies; Myositis specific autoantibody