Scleroderma Disease is a chronic systemic autoimmune disorder that primarily affects the connective tissues, causing fibrosis, vascular changes and excessive antibody production. Sleroderma Disease has been previously identified as CREST Syndrome due to the pneumonic based on its common symptoms. These are: Calcinosis, Raynaud’s Phenomenon, Esophageal Dysfunction, Sclerodactility, and Telangiectasias.
There are two main forms of Scleroderma Disease. These are: Limited Systemic Scleroderma and Diffuse Systemic Scleroderma.
Limited Systemic Scleroderma
Limited Systemic Scleroderma generally affects the cutaneous layers and clinically manifests in the limbs, back and face.
Pulmonary Artery Hypertension is a common side affect of CREST Syndrome
Diffuse Systemic Scleroderma
Diffuse Systemic Scleroderma is a much less likely type of scleroderma and also a more rapidly progressive disease process, affecting vital organs, such as the heart, lung, and kidneys.
What is the treatment for Scleroderma Disease? There is no clear medical treatment for scleroderma disease, however medical treatment options often look at treating the symptoms which manifest.
What is the life expectancy of persons who have Scleroderma Disease? The life expectancy of persons who have Scleroderma Disease are actually quite high. People who have limited systemic scleroderma usually have a normal life expectancy.
I treated a 94 year old last week who had a cough that she couldn’t get rid of – this was related to the pulmonary artery hypertesion (commonly associated with Scleroderma Disease), but lived at home, and was otherwise well. She had been diagnosed with Scleroderam Disease in early life and had 3 children and was still able to walk to the local shops!