Pulmonary arterial hypertension is a serious condition for which there are treatments but no cure, according to the National Heart, Lung, and Blood Institute. Secondary pulmonary arterial hypertension can be caused by or occurs because of another secondary condition. Pulmonary hypertension is associated with a number of rheumatic conditions including:
- Scleroderma (CREST)
- Lupus (Systemic Lupus Erythematosus)
- Rheumatoid Arthritis
"Patients with pulmonary arterial hypertension have elevated levels of endothelin, a potent blood vessel constrictor, in their plasma and lung tissue. Tracleer blocks the binding of endothelin to its receptors, thereby negating endothelin's deleterious effects," according to CenterWatch. In the article, Pulmonary Arterial Hypertension in Scleroderma: A New Treatment, M. Kari Connolly, M.D. reports, Tracleer (bosentan) has advantages over other treatments because it can be taken by mouth, and is generally well tolerated. Additional studies are currently being done to evaluate its effects in other aspects of scleroderma. In a small trial of four patients with systemic sclerosis (Rheumatology Oxford 2004 Nov; 43:1454-6), bosentan successfully treated severe Raynaud phenomenon.
- Guide to Scleroderma
- Guide to Systemic Lupus
- Guide to Rheumatoid Arthritis
- Guide to Raynaud's Phenomenon
- What is Rheumatoid Lung Disease?
- Rheumatic Diseases Associated With Pulmonary Arterial Hypertension
1)"Drugs Approved by the FDA." Drug Name: Tracleer (bosentan). June 29, 2004. CenterWatch Newly Approved Drug Therapies Listing.
2)"Pulmonary Arterial Hypertension." What Is Pulmonary Arterial Hypertension?. August 2006. National Heart, Lung, and Blood Institute.
3)Connolly, M.D., Kari M.. Pulmonary Arterial Hypertension in Scleroderma: A New Treatment. originally published in Scleroderma Voice, 2002 #2. Scleroderma Foundation.
4)"Summary and Comment." Bosentan -- A New Oral Therapy for Severe Raynaud Phenomenon . Published in Journal Watch Dermatology January 18, 2005 . Massachusetts Medical Society
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