ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 619

Survival and Prognostic Factors In Patients With Connective Tissue Disease Associated Pulmonary Arterial Hypertension: Results From Korean Nationwide Registry

Kwi Young Kang1, Chan Hong Jeon2, Sung Jae Choi3, Seung-Ki Kwok4, Seong-Kyu Kim5, Hyoun-Ah Kim6, Eon Jeong Nam7, Yong-Beom Park8, Kichul Shin9, Jaejoon Lee10, Chang-Hoon Lee11, Chan-Bum Choi12, Shin-Seok Lee13 and Dae-Hyun Yoo14, 1Rheumatology, Internal Medicine, Catholic University of Korea, Incheon St. Mary's Hospital, Seoul, South Korea, 2Internal Medicine, Soonchunhyang University College of Medicine, Bucheon, South Korea, 3Rheumatology, Korea University Medical Center, Seoul, South Korea, 4Division of Rheumatology, Department of Internal Medicine, School of Medicine, The Catholic University of Korea, Seoul St. Mary's Hospital, Seoul, South Korea, 5Catholic University of Daegu School of Medicine, Daegu, South Korea, 6Department of Rheumatology, Ajou University School of Medicine, Suwon, South Korea, 7Internal Medicine (Rheumatology), Kyungpook National University School of Medicine, Daegu, South Korea, 8Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea, 9Rheumatology, College of Medicine, Seoul National University, Seoul, South Korea, 10Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea, 11Department of Internal Medicine, School of Medicine, Wonkwang University, Iksan, Chonbuk, South Korea, 12Department of Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Clinical Research Center for Rheumatoid Arthritis (CRCRA), Seoul, South Korea, 13Dept of Int Med/Rheumatology, Chonnam National University Medical School, Gwangju, South Korea, 14Rheumatology, Hanyang University Hospital for Rheumatic Diseases, Seoul, South Korea

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: , , ,

Session Information

Title: Systemic Lupus Erythematosus - Clinical Aspects I - Renal, Malignancy, Cardiovascular Disease

Session Type: Abstract Submissions (ACR)

Background/Purpose: Pulmonary arterial hypertension (PAH) is a major cause of mortality in connective tissue disease (CTD). We sought to quantify survival and determine factors predictive of mortality in a registry of Korean patients with CTD-associated PAH (CTD-PAH), Registry of Pulmonary Hypertension Associated with Rheumatic Disease (REOPARD).

Methods: Patients with CTD-PAH were enrolled between April 2008 and December 2012. Hemodynamic parameters and clinical data such as demographics, WHO functional class, underlying disease, organ involvement, laboratory tests and current treatment were investigated. Follow-up data of enrolled patients were recorded at the 5 year. Survival rate was calculated by the Kaplan–Meier method and the log-rank test. Factors associated with survival were examined by Cox proportional hazards regression analysis.

Results: : One hundred seventy four incident cases were diagnosed by a right heart catheterization or a doppler echocardiograpy. Among 174 patients (61 (35%) with SLE, 50 (29%) with systemic sclerosis, 10 (6%) with MCTD, 22 (13%) with RA), during 3.8 ± 2.7 (mean ± SD) years of follow-up from PAH diagnosis, there were 25 (14%) deaths. One- and 3-year survival rates were 90.7 and 87.3%. Survival was worse for patients with RA-PAH (3-yr survival, 56%; p=0.022). In multiple regression analysis, Low DLCO (p=0.008), pleural effusion (p=0.04), and DM (p=0.009) were poor prognostic factors and anti-UI RNP antibody was a protective factor of mortality (p=0.022). In patients with WHO-FC III/IV, the survival rates were significantly better in patients were received with vasodilators than in patients were not (p=0.038).

Conclusion: In this study among Korean CTD-PAH patients, three –year survival was 87%. Low DLCO, pleural effusion, and DM were independent poor prognostic factors. Anti-UI RNP antibody was a protective factor of mortality. The prompt PAH specific vasodilator therapy may improve survival in patients with severe CTD-PAH.

Disclosure:

K. Y. Kang,
None;

C. H. Jeon,
None;

S. J. Choi,
None;

S. K. Kwok,
None;

S. K. Kim,
None;

H. A. Kim,
None;

E. J. Nam,
None;

Y. B. Park,
None;

K. Shin,
None;

J. Lee,
None;

C. H. Lee,
None;

C. B. Choi,
None;

S. S. Lee,
None;

D. H. Yoo,
None.

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