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Pulmonary hypertension associated with rheumatic diseases: baseline characteristics from the K orean registry
Author(s) -
Jeon Chan Hong,
Chai JiYoung,
Seo YoungIl,
Jun JaeBum,
Koh EunMi,
Lee SooKon
Publication year - 2012
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/j.1756-185x.2012.01815.x
Subject(s) - medicine , mixed connective tissue disease , pulmonary hypertension , rheumatoid arthritis , cardiology , connective tissue disease , cardiac catheterization , blood pressure , hemodynamics , disease , autoimmune disease
Abstract Objectives The RE gistry of P ulmonary H ypertension A ssociated with R heumatic D isease ( REOPARD ) was established in K orea. 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 N ew Y ork H eart A ssociation 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%). Conclusion 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.