title: Antisynthetase syndrome with anti-Jo1 antibodies in 48 patients: pulmonary involvement predicts disease-modifying antirheumatic drug use.
abstract: OBJECTIVE
  To analyze the characteristics, outcomes, and predictive factors of disease-modifying antirheumatic drug (DMARD) use in 48 patients with antisynthetase syndrome [characterized by myositis, interstitial lung disease (ILD), arthritis, Raynaud's phenomenon (RP), and/or mechanic's hands] and the presence of anti-histidyl-transfer RNA synthetase (anti-Jo1) autoantibodies.
  
  
  METHODS
  Forty-eight patients (33 women, 15 men) who were anti-Jo1-positive referred to one center between 1998 and 2008 were analyzed retrospectively.
  
  
  RESULTS
  The median age of disease onset was 43 years [interquartile range (IQR) 33-53 yrs]. The median followup was 5 years (IQR 2-8 yrs). At diagnosis, 81% of patients presented with myositis, 80% ILD, 77% arthralgia, 48% RP, and 21% mechanic's hands. During the followup, 14 patients (29%) had no need for DMARD, while 34 (71%) required DMARD. Patients with mechanic's hands (p=0.02) and higher creatine phosphokinase at diagnosis (median 6070 IU/l vs 1121 IU/l; p=0.002) were more likely to need DMARD. ILD, noted on computed tomography scan by a nonspecific interstitial pneumonia score, was lower in the group of patients with no DMARD need (4 vs 7; p=0.04). Twenty patients (44%) presented with a pulmonary aggravation (worsening of radiologic score of ILD and/or pulmonary function test results) leading to DMARD use. Nonspecific interstitial pneumonia score (7 vs 5; p=0.05) and total lung volume (57.5% vs 70%; p=0.02) values predicted pulmonary aggravation.
  
  
  CONCLUSIONS
  Our study outlines the burden of chest involvement for the prognosis of antisynthetase syndrome in terms of patients' requirement for DMARD therapy.
authors.affiliation: Department of Internal Medicine, Institute of Myology, Université Pierre et Marie Curie, Assistance Publique-Hôpitaux de Paris, Service de Médecine Interne 1, Groupe Hospitalier Pitié-Salpêtrière, 47-83 boulevard de l'Hôpital, 75651 Paris Cedex 13, France.
authors.forename: Raluca
authors.initials: R
authors.lastname: Stanciu
authors.forename: Marguerite
authors.initials: M
authors.lastname: Guiguet
authors.forename: Lucile
authors.initials: L
authors.lastname: Musset
authors.forename: Diane
authors.initials: D
authors.lastname: Touitou
authors.forename: Catherine
authors.initials: C
authors.lastname: Beigelman
authors.forename: Aude
authors.initials: A
authors.lastname: Rigolet
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.forename: Yves
authors.initials: Y
authors.lastname: Allenbach
authors.forename: Baptiste
authors.initials: B
authors.lastname: Hervier
authors.forename: Odile
authors.initials: O
authors.lastname: Dubourg
authors.forename: Thierry
authors.initials: T
authors.lastname: Maisonobe
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Charuel
authors.forename: Anthony
authors.initials: A
authors.lastname: Behin
authors.forename: Serge
authors.initials: S
authors.lastname: Herson
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Philippe
authors.initials: P
authors.lastname: Grenier
authors.forename: Olivier
authors.initials: O
authors.lastname: Benveniste
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000276:immunology*; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D001168:Arthritis / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology; D001323:Autoantibodies; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging*; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D009220:Myositis / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000276:immunology; D011859:Radiography; D012129:Respiratory Function Tests; D012189:Retrospective Studies
chemical_list: D000974:Antibodies, Antinuclear; D018501:Antirheumatic Agents; D001323:Autoantibodies; C046750:Jo-1 antibody