Abstract | OBJECTIVE: METHODS: Twelve patients with PAH related to systemic sclerosis (8 cases), SLE (2 cases), mixed connective tissue disease (1 case) and polymyositis (1 case) attending the Rheumatology Unit of Padova University were treated with bosentan for two years. Distance walked in 6 minutes, right ventricular systolic pressure and mean pulmonary artery pressure estimated by doppler echocardiography were evaluated at baseline and after 6, 12, 18 and 24 months of treatment. Safety was assessed by laboratory tests performed every two months. RESULTS: During bosentan treatment, a significant decrease of right ventricular systolic pressure was observed after 6, 12, 18 and 24 months in comparison to baseline, whereas pulmonary artery mean pressure remained unchanged. Distance walked in 6 minutes slightly increased after 6 and 12 months, but significantly decreased after 18 and 24 months, mostly because complications of CTD which compromised the ability to walk arose in 4 patients. Adverse events related to bosentan were observed in 2 cases. CONCLUSIONS:
Bosentan has been demonstrated effective in reducing pulmonary arterial pressure in a two-year period of treatment. Exercise capacity improved only in the first year of therapy and worsened thereafter, suggesting the opportunity of a combination therapy for a long-term treatment of PAH related to CTD.
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Authors | H Marotta, R Montisci, F Tiso, S Pontarollo, M Rizzo, F Tona, S Iliceto, F Cozzi |
Journal | Reumatismo
(Reumatismo)
2007 Oct-Dec
Vol. 59
Issue 4
Pg. 299-303
ISSN: 0048-7449 [Print] Italy |
Vernacular Title | Due anni di terapia con bosentan dell'ipertensione arteriosa polmonare associata a connettiviti sistemiche. |
PMID | 18157286
(Publication Type: Journal Article)
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Chemical References |
- Antihypertensive Agents
- Sulfonamides
- Bosentan
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Topics |
- Adult
- Aged
- Antihypertensive Agents
(therapeutic use)
- Bosentan
- Connective Tissue Diseases
(complications, physiopathology)
- Echocardiography, Doppler
- Exercise Tolerance
- Female
- Humans
- Hypertension, Pulmonary
(diagnostic imaging, drug therapy, etiology, physiopathology)
- Male
- Middle Aged
- Polymyositis
(complications)
- Scleroderma, Systemic
(complications)
- Sulfonamides
(therapeutic use)
- Treatment Outcome
- Ventricular Function, Right
(drug effects)
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