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

• Published in: International journal of rheumatic diseases 2017-09, Vol.20 (9), p.1227-1236
• Main Authors: Kang, Kwi Young, Jeon, Chan Hong, Choi, Sung Jae, Yoon, Bo Young, Choi, Chan‐Bum, Lee, Chang Hoon, Suh, Chang‐Hee, Lee, Choong Won, Cho, Chul Soo, Nam, Eon Jeong, Koh, Eun‐Mi, Kim, Ho‐Youn, Choi, Hyo Jin, Kim, Hyoun‐Ah, Jun, Jae‐Bum, Lee, Jaejoon, Kim, Jinseok, Ji, Jong Dae, Min, Jun Ki, Kim, Ki Jo, Shin, Kichul, So, Min Wook, Kwon, Seong Ryul, Kim, Seong‐Kyu, Nah, Seong‐Su, Kwok, Seung‐Ki, Lee, Soo‐Kon, Lee, Sung Won, Park, Sung‐Hwan, Park, Won, Park, Yong‐Beom, Lee, Young Ho, Lee, Shin‐Seok, Yoo, Dae Hyun
• Format: Article
• Language: English
• Subjects: Adult; Aged; Antibodies, Antinuclear - blood; Antihypertensive Agents - therapeutic use; Biomarkers - blood; Carbon monoxide; connective tissue disease; Connective Tissue Diseases - diagnosis; Connective Tissue Diseases - epidemiology; Connective Tissue Diseases - mortality; Diabetes; Diabetes mellitus; Diabetes Mellitus - epidemiology; Doppler effect; Echocardiography; Echocardiography, Doppler; Female; Health risk assessment; Humans; Hypertension; Hypertension, Pulmonary - diagnostic imaging; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - epidemiology; Hypertension, Pulmonary - mortality; Incidence; Kaplan-Meier Estimate; Lung diseases; Male; Medical prognosis; Middle Aged; Mortality; Multiple regression analysis; Pleural effusion; Pleural Effusion - epidemiology; Predictive Value of Tests; Prognosis; Proportional Hazards Models; Pulmonary Diffusing Capacity; Pulmonary hypertension; Registries; Regression analysis; Republic of Korea - epidemiology; Rheumatoid arthritis; Risk Factors; Survival; Systemic lupus erythematosus; Systemic sclerosis; Time Factors; Vasodilator Agents - therapeutic use; Vasodilators
• ISSN: 1756-1841; 1756-185X
• DOI: 10.1111/1756-185X.12645

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.