Scleroderma and PAH

Connective Tissue Disease and PAH

06.07.2018

Source: Connections - The Official Magazine of the Canadian PH Community

Scleroderma is a progressive chronic disease that causes hardening and thickening of the skin and internal organs. Scleroderma is an autoimmune disease, meaning that the body’s immune system attacks its own tissues, resulting in inflammation and damage. In scleroderma, the body produces an excess amount of collagen (a type of protein), which can lead to scarring of the skin, tissues under the skin (including muscles and bones), and internal organs, in addition to affecting blood flow throughout the body. The factors that cause this excess production of collagen remain unknown.

Scleroderma can impact people differently, with varying degrees of severity and complications depending on whether the disease is localized (affecting areas of the skin and nearby tissues) or systemic (affecting not only the skin, but also blood vessels and major internal organs). Virtually all persons with systemic scleroderma, also known as systemic sclerosis (SSc), have some loss of lung function, often in the form of pulmonary fibrosis and/or PAH. Unfortunately, those living with SSc have an increased risk of developing PAH because of the significant fibrosis (scarring) of their lungs, which can in turn reduce blood oxygen levels and cause a reflex of increased blood pressure in the pulmonary arteries.

It is estimated that 12% of all patients with systemic scleroderma develop PAH, a type of PAH know as SSc-associated PAH (SSc-PAH). Sadly, PAH is one of the leading causes of death in people living with scleroderma. Studies have also shown that SSc-PAH patients have a poorer response to therapy and significantly worse survival compared to patients with idiopathic PAH (PAH with no known cause). However, since 1996, survival rates have been on the rise thanks to treatment innovations and increased awareness of SSc-PAH.

Scleroderma Canada 

Scleroderma-Society-Canada-Societe-Canadienne-de-la-Sclerodermie.gifScleroderma Canada serves as an advocate nationally for those affected by the disease and works collaboratively with regional scleroderma organizations and the international scleroderma community to achieve common objectives. Scleroderma Canada is committed to promoting public awareness, supporting those affected by scleroderma, and funding research to find a cure. PHA Canada and Scleroderma Canada are partners in the Take Action PAH campaign, which advocates for PAH patients in Canada to have publicly funded access to all Health Canada approved treatments (www.takeactionpah.ca). 

To learn more, visit: www.scleroderma.ca

 

Scleroderma Awareness Month

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To find out more, or get involved with Scleroderma Awareness Month, visit www.sclerodermaaware.org

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