title: Survival and prognostic factors in patients with connective tissue disease-associated pulmonary hypertension diagnosed by echocardiography: results from a Korean nationwide registry.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Internal Medicine, Incheon Saint Mary's Hospital, Catholic University of Korea, Incheon, Korea.
authors.forename: Kwi Young
authors.initials: KY
authors.lastname: Kang
authors.affiliation: Department of Internal Medicine, Hospital Bucheon, Soonchunhyang University, Bucheon, Korea.
authors.forename: Chan Hong
authors.initials: CH
authors.lastname: Jeon
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, College of Medicine, Korea University, Seoul, Korea.
authors.forename: Sung Jae
authors.initials: SJ
authors.lastname: Choi
authors.affiliation: Department of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, Korea.
authors.forename: Bo Young
authors.initials: BY
authors.lastname: Yoon
authors.affiliation: Department of Internal Medicine, College of Medicine, Hanyang University, Seoul, Korea.
authors.forename: Chan-Bum
authors.initials: CB
authors.lastname: Choi
authors.affiliation: Department of Internal Medicine, Wonkwang University College of Medicine, Iksan, Korea.
authors.forename: Chang Hoon
authors.initials: CH
authors.lastname: Lee
authors.affiliation: Department of Rheumatology, Ajou University School of Medicine, Suwon, Korea.
authors.forename: Chang-Hee
authors.initials: CH
authors.lastname: Suh
authors.affiliation: Department of Internal Medicine, Wallace Memorial Baptist Hospital, Busan, Korea.
authors.forename: Choong Won
authors.initials: CW
authors.lastname: Lee
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Chul Soo
authors.initials: CS
authors.lastname: Cho
authors.affiliation: Department of Internal Medicine, Kyungpook National University School of Medicine, Daegu, Korea.
authors.forename: Eon Jeong
authors.initials: EJ
authors.lastname: Nam
authors.affiliation: Department of Internal Medicine, Sungkyunkwan University, Seoul, Korea.
authors.forename: Eun-Mi
authors.initials: EM
authors.lastname: Koh
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Ho-Youn
authors.initials: HY
authors.lastname: Kim
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea.
authors.forename: Hyo Jin
authors.initials: HJ
authors.lastname: Choi
authors.affiliation: Department of Rheumatology, Ajou University School of Medicine, Suwon, Korea.
authors.forename: Hyoun-Ah
authors.initials: HA
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, College of Medicine, Hanyang University, Seoul, Korea.
authors.forename: Jae-Bum
authors.initials: JB
authors.lastname: Jun
authors.affiliation: Department of Internal Medicine, Sungkyunkwan University, Seoul, Korea.
authors.forename: Jaejoon
authors.initials: J
authors.lastname: Lee
authors.affiliation: Department of Internal Medicine, Jeju National University School of Medicine, Jeju, Korea.
authors.forename: Jinseok
authors.initials: J
authors.lastname: Kim
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, College of Medicine, Korea University, Seoul, Korea.
authors.forename: Jong Dae
authors.initials: JD
authors.lastname: Ji
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Jun Ki
authors.initials: JK
authors.lastname: Min
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Ki Jo
authors.initials: KJ
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Borame Hospital, Seoul National University, Seoul, Korea.
authors.forename: Kichul
authors.initials: K
authors.lastname: Shin
authors.affiliation: Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Min Wook
authors.initials: MW
authors.lastname: So
authors.affiliation: Departments of Internal Medicine, Inha University College of Medicine, Incheon, Korea.
authors.forename: Seong Ryul
authors.initials: SR
authors.lastname: Kwon
authors.affiliation: Department of Internal Medicine, Catholic University of Daegu, Daegu, Korea.
authors.forename: Seong-Kyu
authors.initials: SK
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan, Korea.
authors.forename: Seong-Su
authors.initials: SS
authors.lastname: Nah
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Seung-Ki
authors.initials: SK
authors.lastname: Kwok
authors.affiliation: Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Soo-Kon
authors.initials: SK
authors.lastname: Lee
authors.affiliation: Department of Internal Medicine, College of Medicine, Dong-A University, Pusan, Korea.
authors.forename: Sung Won
authors.initials: SW
authors.lastname: Lee
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
authors.forename: Sung-Hwan
authors.initials: SH
authors.lastname: Park
authors.affiliation: Departments of Internal Medicine, Inha University College of Medicine, Incheon, Korea.
authors.forename: Won
authors.initials: W
authors.lastname: Park
authors.affiliation: Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Yong-Beom
authors.initials: YB
authors.lastname: Park
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, College of Medicine, Korea University, Seoul, Korea.
authors.forename: Young Ho
authors.initials: YH
authors.lastname: Lee
authors.affiliation: Department of Rheumatology, Chonnam National University Medical School, Kwangju, Korea.
authors.forename: Shin-Seok
authors.initials: SS
authors.lastname: Lee
authors.affiliation: Department of Internal Medicine, College of Medicine, Hanyang University, Seoul, Korea.
authors.forename: Dae Hyun
authors.initials: DH
authors.lastname: Yoo
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood; D000959:Antihypertensive Agents / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality; D003920:Diabetes Mellitus / Q000453:epidemiology; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D015994:Incidence; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D010996:Pleural Effusion / Q000453:epidemiology; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D011653:Pulmonary Diffusing Capacity; D012042:Registries; D056910:Republic of Korea / Q000453:epidemiology; D012307:Risk Factors; D013997:Time Factors; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000974:Antibodies, Antinuclear; D000959:Antihypertensive Agents; D015415:Biomarkers; D014665:Vasodilator Agents
keywords: connective tissue disease; pulmonary hypertension; survival; systemic lupus erythematosus; systemic sclerosis