Saturday, January 29, 2011

What is Scleroderma?


Scleroderma is an autoimmune disease which means that it is a condition in which the body’s immune system attacks its own tissues. The normal role of the immune system is to provide protection from invaders such as viruses. In autoimmune disorders, this ability to distinguish foreign from self is compromised. As immune cells attack the body’s own tissue, inflammation and damage result. Scleroderma (the name means “hard skin”) can vary a great deal in terms of severity. For some, it is a mild condition; for others it can be life-threatening. Although there are medications to slow down disease progression and help with symptoms, there is as yet no cure for scleroderma.

There are 2 types of Scleroderma and I have Systemic Sclerosis.

Several clinical features occur in Systemic Sclerosis.

Raynaud’s phenomenon is a condition in which the fingers turn pale or blue upon cold exposure, and then become ruddy or red upon warming up. These episodes are caused by a spasm of the small blood vessels in the fingers. As time goes on, these small blood vessels become damaged to the point that they are totally blocked. This can lead to ulcerations of the fingertips. 

Esophagus - The esophagus is also affected in Systemic Sclerosis patients with loss of the usual movement. As a result, food can “hang up” in the esophagus, and stomach acid can reflux, causing heartburn. Some people have telangiectasias in the esophagus, stomach, and bowel that can be a source of bleeding.

Lungs - People with Systemic Sclerosis are at risk of developing pulmonary fibrosis (scar tissue in the lungs that interferes with breathing, also called interstitial lung disease), kidney disease, and bowel disease. Symptoms of pulmonary disease include a dry cough and shortness of breath. However, in the early stages there may not be any symptoms at all. Pulmonary hypertension (PH) is high blood pressure in the blood vessels of the lungs. It is totally independent of the usual blood pressure that is taken in the arm. This tends to develop in patients with Systemic Slerosis after several years of disease. The most common symptom is shortness of breath on exertion. However, several tests need to be done to determine if PH is the real culprit. If the ultrasound of the heart, called Doppler echocardiogram,
is abnormal, then a right heart catheterization needs to be done to actually measure the pressure in
the lung and to test for other abnormalities that can cause PH. Because there are now many medications to treat PH, the earlier it is detected and treated, the better the result will be.

Kidneys - Kidney involvement occurs frequently in Systemic Sclerosis, especially in the first five years post-diagnosis, and typically takes the form of a sudden increase in blood pressure. As is the
case with usual high blood pressure, there are no symptoms at first. However, if undetected and untreated, this high blood pressure can damage the kidneys in a matter of weeks, which is why it is called scleroderma renal crisis. The key to management and prevention of permanent kidney damage is early detection and treatment with ACE inhibitors.

Gut - The risk of extensive gut involvement, with slowing of the movement or motility of the stomach and bowel, is higher in those with Systemic Sclerosis. Symptoms include feeling bloated after eating, diarrhea, or alternating diarrhea and constipation.

Other Skin Symptoms - Calcinosis refers to the presence of calcium deposits in, or just under, the skin. This takes the form of firm nodules or lumps that tend to occur on the fingers or forearms, but can occur anywhere on the body.




This information was gathered from http://www.scleroderma.org/.





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