Intravenous epoprostenol treatment of patients with connective tissue disease and pulmonary arterial hypertension at a single center.
OBJECTIVE
To assess the efficacy of epoprostenol treatment in Japanese patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD).


METHODS
Sixteen patients with PAH-CTD treated with continuous intravenous epoprostenol at a single center between 2000 and 2009 were enrolled. Baseline characteristics, short-term and long-term outcomes, predictors of mortality, and safety profiles were evaluated. For survival analysis, 16 controls were selected who matched the underlying CTD, World Health Organization functional class, and use of PAH drugs, except for epoprostenol.


RESULTS
Six patients had systemic lupus erythematosus, five had mixed CTD, four had systemic sclerosis, and one had primary Sjögren's syndrome. The mean pulmonary arterial pressure (mPAP), cardiac index (CI), pulmonary vascular resistance, and functional class were significantly improved during the first 6 months of epoprostenol treatment. Cumulative survival rates at 1, 2, and 3 years in epoprostenol-treated patients were 69, 69, and 55%, respectively, and were significantly better than those of the controls. Functional class, CI at baseline, and reduction of mPAP at 6 months were identified as predictors of survival. Adverse events, including flushing and catheter-related infection, were frequent, but all patients tolerated the treatment.


CONCLUSIONS
Based on the improvements in both short-term and long-term outcomes among our patient cohort, epoprostenol is an effective treatment for CTD patients with advanced PAH.
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
Yuichiro
Y
Shirai
Hidekata
H
Yasuoka
Tsutomu
T
Takeuchi
Toru
T
Satoh
Masataka
M
Kuwana
D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D003240:Connective Tissue Diseases / Q000150:complications*; D011464:Epoprostenol / Q000008:administration & dosage / Q000627:therapeutic use*; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy*; D007262:Infusions, Intravenous; D008875:Middle Aged; D016896:Treatment Outcome
D000959:Antihypertensive Agents; D011464:Epoprostenol