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Patients with non-Jo-1 anti-tRNA-synthetase autoantibodies have worse survival than Jo-1 positive patients
  1. Rohit Aggarwal1,
  2. Elaine Cassidy1,
  3. Noreen Fertig1,
  4. Diane Carol Koontz1,
  5. Mary Lucas1,
  6. Dana P Ascherman2,
  7. Chester V Oddis1
  1. 1Department of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
  2. 2Department of Medicine, Division of Rheumatology, University of Miami, Miami, Florida, USA
  1. Correspondence to Dr Rohit Aggarwal, Department of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine, Falk Medical Building, Suite 2B, 3601 Fifth Avenue, Pittsburgh, PA 15213, USA;aggarwalr{at}upmc.edu
  2. RA and EC contributed equally to the manuscript and are co-first authors.

Abstract

Objective To compare the cumulative survival and event free survival in patients with Jo-1 versus non-Jo-1 anti-tRNA synthetase autoantibodies (anti-synAb).

Methods Anti-synAb positive patients initially evaluated from 1985 to 2009 were included regardless of the connective tissue disease (CTD) diagnosis. Clinical data were extracted from a prospectively collected database and chart review. Survival between Jo-1 and non-Jo-1 was compared by log rank and Cox proportional hazards methods.

Results 202 patients possessed anti-synAb: 122 Jo-1 and 80 non-Jo-1 (35 PL-12; 25 PL-7; 9 EJ; 6 KS; 5 OJ). The diagnoses at first visit for Jo-1 and non-Jo-1 patients were myositis in 83% and 40.0%, overlap or undifferentiated CTD in 17% and 47.5%, and systemic sclerosis in 0% and 12.5%, respectively (p<0.001). The median delay in diagnosis was 0.4 years in Jo-1 patients versus 1.0 year in non-Jo-1 patients (p<0.001). The most common causes of death in the overall cohort were pulmonary fibrosis in 49% and pulmonary hypertension in 11%. The 5- and 10-year unadjusted cumulative survival was 90% and 70% for Jo-1 patients, and 75% and 47% for non-Jo-1 patients (p<0.005). The hazard ratio (HR) of non-Jo-1 patients compared with Jo-1 patients was 1.9 (p=0.01) for cumulative and 1.9 (p=0.008) for event free survival from diagnosis. Age at first diagnosis and diagnosis delay but not gender, ethnicity and CTD diagnosis influenced survival.

Conclusions Non-Jo-1 anti-synAb positive patients have decreased survival compared with Jo-1 patients. The difference in survival may be partly attributable to a delay in diagnosis in the non-Jo-1 patients.

  • Dermatomyositis
  • Polymyositis
  • Outcomes research
  • Autoantibodies

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