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AB0625 Survival and Prognostic Factors in Patients with Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: Results from A Korean Nationwide Registry
  1. K.Y. Kang1,
  2. C.H. Jeon2,
  3. S.J. Choi3,
  4. B.Y. Yoon4,
  5. C.-B. Choi5,
  6. C.H. Lee6,
  7. C.-H.S. Suh7,
  8. C.W. Lee8,
  9. C.S.C. Cho9,
  10. E.J. Nam10,
  11. E.-M. Koh11,
  12. H.-Y. Kim12,
  13. H.J. Choi13,
  14. H.-A. Kim7,
  15. J.-B. Jun5,
  16. J. Lee11,
  17. J. Kim14,
  18. J.D. Ji3,
  19. J.K. Min12,
  20. K.J. Kim12,
  21. K. Shin15,
  22. M.W. So16,
  23. S.R. Kwon17,
  24. S.-K. Kim18,
  25. S.-S. Nah19,
  26. S.-K. Kwok12,
  27. S.-K. Lee20,
  28. S.W. Lee21,
  29. S.-H. Park12,
  30. W. Park17,
  31. Y.-B. Park20,
  32. Y.H.L. Lee3,
  33. S.-S. Lee22,
  34. D.H. Yoo5
  1. 1Catholic university of Korea, Seoul
  2. 2Department of Internal Medicine, Hospital Bucheon, Soonchunhyang University, Bucheon
  3. 3Division of Rheumatology, Department of Internal Medicine, College of Medicine, Korea University, Seoul
  4. 4Departments of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, Ilsan
  5. 5Department of Internal Medicine, College of Medicine, Hanyang University, Seoul
  6. 6Departments of Internal Medicine, Wonkwang University College of Medicine, Iksan
  7. 7Department of Rheumatology, Ajou University School of Medicine, Suwon
  8. 8Department of Internal Medicine, Wallace Memorial Baptist Hospital, Busan
  9. 9Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul
  10. 10Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu
  11. 11Department of Internal Medicine, Sungkyunkwan University
  12. 12Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul
  13. 13Division of Rheumatology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Seoul
  14. 14Departments of Internal Medicine, Jeju National University School of Medicine, Jeju
  15. 15Department of Internal Medicine, Seoul National University, Borame Hospital
  16. 16Department of Internal Medicine, Asan Medical Center, University of Ulsan college of Medicine, Seoul
  17. 17Departments of Internal Medicine, Inha University College of Medicine, Incheon
  18. 18Department of Internal Medicine, Catholic University of Daegu, Daegu
  19. 19Department of Internal Medicine, Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan
  20. 20Department of Internal Medicine, Yonsei University College of Medicine, Seoul
  21. 21Department of Internal Medicine, College of Medicine, Dong-A University, Pusan
  22. 22Department of Rheumatology, Chonnam National University Medical School, Kwangju, Korea, Republic Of


Background Pulmonary arterial hypertension (PAH) is a major cause of mortality in connective tissue disease (CTD).

Objectives The survival rates and mortality-predictive factors of a nationwide registry of Korean patients with CTD-PAH were determined.

Methods Patients with CTD-PAH were enrolled between April 2008 and December 2012. Hemodynamic parameters and clinical data (WHO-functional class [FC], organ involvement, laboratory tests, and treatment agents) were recorded. Survival rates were calculated by using the Kaplan–Meier method. Mortality-associated factors were examined by Cox proportional hazards regression analysis.

Results In total, 174 incident PAH cases (61 with systemic lupus erythematosus, 50 with systemic sclerosis, 10 with mixed CTD, 22 with rheumatoid arthritis [RA], and 31 with other CTDs) were diagnosed by right heart catheterisation or Doppler echocardiography. Of these, 25 (14%) died during the 3.8±2.7 year follow-up period after PAH diagnosis. The 1 and 3 year survival rates were 90.7% and 87.3%, respectively. Compared to the other CTD-PAHs, RA-PAH had the lowest survival rates (56% 3 year survival; p=0.022). Multiple regression analysis revealed that low DLCO, pleural effusion, and diabetes mellitus were poor prognostic factors (p=0.008, 0.04, and 0.009, respectively). Anti-UI-RNP antibody positivity was protective (p=0.022). In patients with WHO-FC III/IV, patients who received vasodilators had lower mortality than those who did not (p=0.038).

Conclusions In Korean patients with CTD-PAH, the 3 year survival rate was 87%. Low DLCO, pleural effusion, and DM were independent poor prognostic factors. Anti-UI-RNP antibody was protective. Prompt PAH-specific vasodilator therapy may improve the survival of patients with severe CTD-PAH.

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.1271

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