Pulmonary hypertension associated with rheumatic diseases: baseline characteristics from the Korean registry.
OBJECTIVE
The REgistry of Pulmonary Hypertension Associated with Rheumatic Disease (REOPARD) was established in Korea. The baseline data are described from the second year of the registry's operation.


METHODS
Patients with a connective tissue disease (CTD) who met the modified definition of the WHO group I pulmonary arterial hypertension (PAH) were enrolled. PAH was defined as a systolic pulmonary arterial pressure> 40 mmHg by echocardiography or mean pulmonary arterial pressure> 25 mmHg by right heart catheterization. Hemodynamic parameters and clinical data such as demographics, functional class, underlying disease, organ involvement, laboratory tests and current treatment were recorded.


RESULTS
A total of 321 patients were enrolled during the 2-year study period from 2008 to 2010. The mean age of the patients at registration was 51.9 years and 87.5% were female. Most patients were diagnosed by echocardiography and only 24 patients (7.5%) underwent cardiac catheterization. Exertional dyspnea was present in 63.6% of patients and 31.8% were New York Heart Association class III or IV. Among the patients, systemic lupus erythematosus accounted for 35.3%, systemic sclerosis 28.3%, rheumatoid arthritis 7.8%, overlap syndrome 9.0%, and mixed connective tissue disease 5.9%. There were no significant differences in hemodynamics, functional class, diffusing capacity and N-terminal pro-brain natriuretic peptide levels between the disease subgroups. Treatments consisted of calcium antagonists (57.0%), endothelin antagonists (32.7%), prostanoids (27.1%), phosphodiesterase-5 inhibitors (14.3%) and combinations (37.4%).


CONCLUSIONS
Compared with previous studies, the results showed some differences: underlying diseases, functional status and treatments. This may be due to differences in ethnic background and diagnostic methods of our study.
Department of Internal Medicine, Soonchunhyang University Hospital Bucheon, Bucheon, Gyeonggi-do, Korea.
Chan Hong
CH
Jeon
Ji-Young
JY
Chai
Young-Il
YI
Seo
Jae-Bum
JB
Jun
Eun-Mi
EM
Koh
Soo-Kon
SK
Lee
D000328:Adult; D000368:Aged; D001172:Arthritis, Rheumatoid / Q000097:blood / Q000453:epidemiology / Q000503:physiopathology; D003625:Data Collection; D005260:Female; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood* / Q000453:epidemiology / Q000503:physiopathology*; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000453:epidemiology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000097:blood / Q000453:epidemiology / Q000503:physiopathology; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D011653:Pulmonary Diffusing Capacity / Q000502:physiology; D012042:Registries; D056910:Republic of Korea / Q000453:epidemiology; D012189:Retrospective Studies; D012216:Rheumatic Diseases / Q000150:complications*; D012595:Scleroderma, Systemic / Q000097:blood / Q000453:epidemiology / Q000503:physiopathology
D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain