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; Sung Jae 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 Kim; Kichul 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

doi:10.1111/1756-185X.12645

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, Sung Jae 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

Research output: Contribution to journal › Article › peer-review

25 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 language	English
Pages (from-to)	1227-1236
Number of pages	10
Journal	International journal of rheumatic diseases
Volume	20
Issue number	9
DOIs	https://doi.org/10.1111/1756-185X.12645
Publication status	Published - 2017 Sept
Externally published	Yes

Bibliographical note

Publisher Copyright:
© 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd

Keywords

connective tissue disease
pulmonary hypertension
survival
systemic lupus erythematosus
systemic sclerosis

ASJC Scopus subject areas

Rheumatology

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1111/1756-185X.12645

Cite this

Kang, K. Y., Jeon, C. H., Choi, S. J., 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

Survival and prognostic factors in patients with connective tissue disease-associated pulmonary hypertension diagnosed by echocardiography: results from a Korean nationwide registry. / Kang, Kwi Young; Jeon, Chan Hong; Choi, Sung Jae et al.
In: International journal of rheumatic diseases, Vol. 20, No. 9, 09.2017, p. 1227-1236.

Research output: Contribution to journal › Article › peer-review

Kang, KY, Jeon, CH, Choi, SJ, Yoon, BY, Choi, CB, Lee, CH, Suh, CH, Lee, CW, Cho, CS, Nam, EJ, Koh, EM, Kim, HY, Choi, HJ, Kim, HA, Jun, JB, Lee, J, Kim, J, Ji, JD, Min, JK, Kim, KJ, Shin, K, So, MW, Kwon, SR, Kim, SK, Nah, SS, Kwok, SK, Lee, SK, Lee, SW, Park, SH, Park, W, Park, YB, Lee, YH, Lee, SS & Yoo, DH 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, vol. 20, no. 9, pp. 1227-1236. https://doi.org/10.1111/1756-185X.12645

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

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.",

keywords = "connective tissue disease, pulmonary hypertension, survival, systemic lupus erythematosus, systemic sclerosis",

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

note = "Publisher Copyright: {\textcopyright} 2015 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd",

year = "2017",

month = sep,

doi = "10.1111/1756-185X.12645",

language = "English",

volume = "20",

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TY - JOUR

T1 - Survival and prognostic factors in patients with connective tissue disease-associated pulmonary hypertension diagnosed by echocardiography

T2 - results from a Korean nationwide registry

AU - Kang, Kwi Young

AU - Jeon, Chan Hong

AU - Choi, Sung Jae

AU - Yoon, Bo Young

AU - Choi, Chan Bum

AU - Lee, Chang Hoon

AU - Suh, Chang Hee

AU - Lee, Choong Won

AU - Cho, Chul Soo

AU - Nam, Eon Jeong

AU - Koh, Eun Mi

AU - Kim, Ho Youn

AU - Choi, Hyo Jin

AU - Kim, Hyoun Ah

AU - Jun, Jae Bum

AU - Lee, Jaejoon

AU - Kim, Jinseok

AU - Ji, Jong Dae

AU - Min, Jun Ki

AU - Kim, Ki Jo

AU - Shin, Kichul

AU - So, Min Wook

AU - Kwon, Seong Ryul

AU - Kim, Seong Kyu

AU - Nah, Seong Su

AU - Kwok, Seung Ki

AU - Lee, Soo Kon

AU - Lee, Sung Won

AU - Park, Sung Hwan

AU - Park, Won

AU - Park, Yong Beom

AU - Lee, Young Ho

AU - Lee, Shin Seok

AU - Yoo, Dae Hyun

PY - 2017/9

Y1 - 2017/9

N2 - 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.

AB - 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.

KW - connective tissue disease

KW - pulmonary hypertension

KW - survival

KW - systemic lupus erythematosus

KW - systemic sclerosis

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U2 - 10.1111/1756-185X.12645

DO - 10.1111/1756-185X.12645

M3 - Article

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AN - SCOPUS:85030786251

SN - 1756-1841

VL - 20

SP - 1227

EP - 1236

JO - International journal of rheumatic diseases

JF - International journal of rheumatic diseases

IS - 9

ER -

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

Abstract

Bibliographical note

Keywords

ASJC Scopus subject areas

UN SDGs

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