Survival and prognostic factors in patients with connective tissue disease-associated pulmonary hypertension diagnosed by echocardiography: results from a Korean nationwide registry

Kwi Young Kang, Chan Hong Jeon, Sungjae Choi, Bo Young Yoon, Chan Bum Choi, Chang Hoon Lee, Chang Hee Suh, Choong Won Lee, Chul Soo Cho, Eon Jeong Nam, Eun Mi Koh, Ho Youn Kim, Hyo Jin Choi, Hyoun Ah Kim, Jae Bum Jun, Jaejoon Lee, Jinseok Kim, Jong Dae Ji, Jun Ki Min, Ki Jo KimKichul Shin, Min Wook So, Seong Ryul Kwon, Seong Kyu Kim, Seong Su Nah, Seung Ki Kwok, Soo Kon Lee, Sung Won Lee, Sung Hwan Park, Won Park, Yong Beom Park, Young Ho Lee, Shin Seok Lee, Dae Hyun Yoo

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7 Citations (Scopus)

Abstract

Objectives: Pulmonary arterial hypertension (PAH) is a major cause of mortality in connective tissue disease (CTD). The survival rates and mortality-predictive factors of a nationwide registry of Korean patients with CTD-PH measured by echocardiography were determined. Methods: Patients with CTD-PH 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 PH 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 Doppler echocardiography. Of these, 25 (14%) died during the 3.8 ± 2.7 year follow-up period after PH diagnosis. The 1- and 3-year survival rates were 90.7% and 87.3%, respectively. Compared to the other CTD-PHs, RA-PH had the lowest survival rates (56% 3 year survival; P = 0.022). Multiple regression analysis revealed that low diffusion capacity of carbon monoxide (DLCO), pleural effusion and diabetes mellitus were poor prognostic factors (P = 0.008, 0.04 and 0.009, respectively). Anti-UI-RNP (ribonucleoprotein) 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-PH, the 3-year survival rate was 87%. Low diffusion capacity of carbon monoxide (DLCO), pleural effusion and diabetes mellitus 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-PH.

Original languageEnglish
Pages (from-to)1227-1236
Number of pages10
JournalInternational Journal of Rheumatic Diseases
Volume20
Issue number9
DOIs
Publication statusPublished - 2017 Sep 1

Keywords

  • connective tissue disease
  • pulmonary hypertension
  • survival
  • systemic lupus erythematosus
  • systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology

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    Kang, K. Y., Jeon, C. H., Choi, S., Yoon, B. Y., Choi, C. B., Lee, C. H., Suh, C. H., Lee, C. W., Cho, C. S., Nam, E. J., Koh, E. M., Kim, H. Y., Choi, H. J., Kim, H. A., Jun, J. B., Lee, J., Kim, J., Ji, J. D., Min, J. K., ... Yoo, D. H. (2017). Survival and prognostic factors in patients with connective tissue disease-associated pulmonary hypertension diagnosed by echocardiography: results from a Korean nationwide registry. International Journal of Rheumatic Diseases, 20(9), 1227-1236. https://doi.org/10.1111/1756-185X.12645