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

Autor: Jun-Ki Min, Chul Soo Cho, Hyoun-Ah Kim, Sung Jae Choi, Young Ho Lee, Min Wook So, Ho-Youn Kim, Seong Su Nah, Bo Young Yoon, Kichul Shin, Chang Hee Suh, Dae Hyun Yoo, Jae-Bum Jun, Yong Beom Park, Eun Mi Koh, Chan-Bum Choi, Seong Ryul Kwon, Soo Kon Lee, Shin-Seok Lee, Jaejoon Lee, Choong Won Lee, Jong Dae Ji, Eon Jeong Nam, Jinseok Kim, Seung Ki Kwok, C.-H. Lee, Hyo Jin Choi, Sung Hwan Park, Chan Hong Jeon, Seong-Kyu Kim, Won Park, Ki Jo Kim, Kwi Young Kang, Sung Won Lee
Rok vydání: 2015
Předmět:
Adult
Male
medicine.medical_specialty
Time Factors
Pleural effusion
Hypertension
Pulmonary
Vasodilator Agents
Kaplan-Meier Estimate
030204 cardiovascular system & hematology
Gastroenterology
03 medical and health sciences
0302 clinical medicine
Rheumatology
Predictive Value of Tests
Risk Factors
DLCO
Internal medicine
Republic of Korea
Diabetes Mellitus
Humans
Medicine
Registries
Connective Tissue Diseases
Survival rate
Antihypertensive Agents
Aged
Proportional Hazards Models
030203 arthritis & rheumatology
business.industry
Proportional hazards model
Incidence
Middle Aged
Prognosis
medicine.disease
Pulmonary hypertension
Connective tissue disease
Echocardiography
Doppler
Surgery
Pleural Effusion
Antibodies
Antinuclear
Rheumatoid arthritis
Predictive value of tests
Pulmonary Diffusing Capacity
Female
business
Biomarkers
Zdroj: International Journal of Rheumatic Diseases. 20:1227-1236
ISSN: 1756-1841
Popis: 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.
Databáze: OpenAIRE
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