Scleroderma, Lupus Information Sheets
Pulmonary Arterial Hypertension
Pulmonary Arterial Hypertension (PAH) is a common condition in scleroderma, lupus and related illnesses, affecting up to 35% of these individuals. As PAH develops, blood flow through the pulmonary arteries is restricted and the right side of the heart is put under increasing strain to pump blood through to the lungs.
The condition is often silent, with the development of clinical symptoms (breathlessness, chest tightness, limited exercise capacity and fatigue) sometimes signaling the presence of advanced disease. This has prompted the development of improved methods for detecting PAH, including annual echocardiographic screening of asymptomatic individuals with systemic autoimmunity.
PAH is no longer greeted with pessimism, with a range of treatments (phosphodiesterase inhibitors, prostacyclin analogues, and endothelin blockade) improving exercise capacity and survival. With these therapeutic developments, a new impetus has been provided for earlier detection of PAH in at-risk populations, with exercise echocardiography being proposed as a useful tool.
The Scleroderma-Lupus Autoimmune Resource Centre, with the support of Actelion Pharmaceuticals, has been offering free screening for PAH to all members of the centre.
To JOIN please download & complete the Membership_Form.pdf
If you like further information on PAH please contact Melisa Eketone via e-mail or
Phone: 02 49214095
Fax: 02 49855347