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FRI0291 Clinical Spectrum Time Course in Non Anti Jo-1 Positive Antisynthetase Syndrome
  1. L. Cavagna1,
  2. H. Andersson2,
  3. M.A. González-Gay3,
  4. O. Molberg2,
  5. F. Franceschini4,
  6. I. Cavazzana5,
  7. S. Castañeda6,
  8. F.J. Lopez Longo7,
  9. S. Balduzzi1,
  10. C. Montecucco1,
  11. K. Triantafyllias8,
  12. J. Weinmann-Menke9,
  13. J. Rojas-Serrano10,
  14. A. Sifuentes Giraldo11,
  15. J. Bachiller-Corral11,
  16. F. Salaffi12,
  17. F. Iannone13,
  18. M. Giannini13,
  19. L. Nuno14,
  20. F. Bonella15,
  21. U. Costabel15,
  22. S. Parisi16,
  23. C. Selmi17,
  24. C. Scirè18,
  25. M. Benucci19,
  26. A. Doria20,
  27. R. Caporali1,
  28. D.I. Pérez-Román10,
  29. A. Ghirardello21,
  30. on behalf of AENEAS Coll. Group
  1. 1Rheumatology, University & Irccs Found. Policlinico S.Matteo, Pavia, Italy
  2. 2Rheumatology, University Hospital, Oslo, Norway
  3. 3Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain
  4. 4Rheumatology, University and AO Spedali Civili
  5. 5Rheumatology, University & AO Spedali Civili, Brescia, Italy
  6. 6Rheumatology, Hospital Universitario de la Princesa
  7. 7Rheumatology, Hospital General Universitario G. Marañόn, Madrid, Spain
  8. 8Rheumatology, ACURA Center, Bad Kreuznach
  9. 9Rheumatology, University Hospital J. Gutenberg, Mainz, Germany
  10. 10ILD and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Tlalpan, Mexico
  11. 11Rheumatology, University Hospital Ramόn y Cajal, Madrid, Spain
  12. 12Rheumatology, University of Marche and C. Urbani Hospital, Jesi
  13. 13Rheumatology, University of Bari, Bari, Italy
  14. 14Rheumatology, Hospital Universitario La Paz, Madrid, Spain
  15. 15Interstitial and Rare Lung Disease Unit, Ruhrlandklinik, University Hospital, University of Duisburg-Essen, Essen, Germany
  16. 16Rheumatology, Città della Salute e della Scienza, Torino
  17. 17Rheumatology, Humanitas Research Hospital, Rozzano
  18. 18Epidemiology Unit, Italian Society for Rheumatology, Milan
  19. 19Rheumatology, Nuovo Osp. S Giovanni di Dio, Florence
  20. 20Rheumatology, University of Padua, Padova
  21. 21Rheumatology, University of Padua, Padua, Italy

Abstract

Background The clinical phenotype of the antisynthetase syndrome (AS) has been related with the underlying autoantibody specificity. The typical disease triad (arthritis, myositis, and interstitial lung disease -ILD) is common in anti Jo-1 + AS, whereas isolated ILD is associated with anti PL-7 and PL-12 Ab. The majority of anti Jo-1 + patients without all triad findings at the onset (incomplete ASSD) will develop lacking features during the followup

Objectives To assess the triad clinical spectrum time course in non anti Jo-1 + AS

Methods The study cohort included patients + for anti-EJ, OJ, PL-7, or PL-12 Ab with at least 1 triad finding. Clinical features were retrospectively collected and analyzed

Results we included 65 non anti Jo-1 + AS (7 anti EJ, 4 anti OJ, 30 anti PL-12, 24 anti PL-7; 49 females, 16 males). In median, age at disease onset was 53 years (IQR 42–64), diagnostic delay 9 months (IQR 3–23.5), followup 60 months (IQR 20.5–113). Patients' characteristics are reported in Table 1. During the followup, 22/61 patients with incomplete AS developed new triad findings, in median 12 months (IQR 6–27) after disease onset. The frequency of incomplete AS developing new triad findings and the length of followup were significantly reduced in non anti Jo-1 + patients with respect to our corresponding anti Jo-1 + cohort (respectively: 22/61 vs 138/220, p<0.001; median 60 months, IQR 18.5–113 vs median 75 months, IQR 39–134; p=0.002)

Table 1

Conclusions In non anti Jo-1 + patients the ex novo occurrence of classic triad finding is common, but less frequent than in anti Jo-1 + patients. The different length of followup may explain at least partly this difference

  1. Cavagna L, et al. Medicine (Baltimore) 2015;94:e1144

Disclosure of Interest None declared

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