Pulmonary Arterial Hypertension And Scleroderma PDF Print E-mail
Wednesday, 11 November 2009 14:42
Scleroderma is a disease that affects the skin and internal organs. The literal meaning of the word is "hard skin"; people with Scleroderma experience a hardening and tightening of their skin. The inflammation and scarring associated with Scleroderma can cause damage to the lungs, and it may lead to a condition called pulmonary arterial hypertension (PAH).

Pulmonary hypertension is a state of high blood pressure in the lungs, and according to the University of Michigan Health System, close to half of people who have Scleroderma face PAH as well.

The most common symptom of PAH is shortness of breath with physical activity. Fainting or near fainting with physical activity may occur as well as other symptoms such as fluid retention and chest discomfort. The diagnosis of PAH is often overlooked by the community physician. It is sufficiently common and of such high impact that all patients with Scleroderma should be screened for its presence on a regular basis.

As with many features of Scleroderma, the basic underlying cause of PAH is the progressive scarring of the inner lining of the small artery. The changes in the lung blood vessels look remarkably similar to those in the fingers, kidneys and gastrointestinal tract. Many in the international research community think of Scleroderma as a blood vessel narrowing disease and view the immune system activation and tissue scarring as secondary events.

Treating PAH involves medication and monitoring of organ function, and can be pursued as outlined below.

Step 1:

Treat pulmonary hypertension associated with Scleroderma with a class of medication called vasodilators. Medications of this kind are used to open blood vessels and can lead to a lowering of the blood pressure. Some drugs used to treat PAH may be given intravenously at a medical facility.

Step 2:
Prevent blood clots in the lungs, one of the dangers of scleroderma-related pulmonary hypertension, with blood thinners and diuretics. Blood thinning medications prevent the blood cells from sticking together. Diuretics promote urination and may also prevent the clotting of the blood.

Step 3:
Block the body's production of endothelin with a medication called bosentan. The Scleroderma Foundation explains that people with PAH have a higher-than-normal level of endothelin in their bodies. Endothelin causes the blood vessels to constrict. Bosentan is a drug specifically approved to treat pulmonary hypertension, but it should not be used by pregnant women due to the risk of birth defects.

Step 4:
Monitor liver function while taking medications to treat PAH. An average of 11 percent of Scleroderma patients show abnormal liver function while undergoing treatment for PAH. Liver function should be tested before a drug regimen begins and on a regular basis during the course of treatment.

For further information on PAH, visit the University of Michigan Health System's page here, and the Scleroderma Foundation's page here.
 
More articles :

» Scleroderma and Pregnancy

In an ideal world, men and women would be able to start their families with no difficulties or problems.  However, there are many issues that can complicate matters and Scleroderma and Pregnancy is one of those issues. It is very important to...

» PUVA Therapy In The Treatment of Localized Scleroderma

In localized , disease activity would tend to stop spontaneously over time. Treatment is important to reduce disabilities caused by the active phases of disease. According to , there are three treatments used in the treatment of localized...

» New Study Shows Increased Risk Of Cancer In Scleroderma

patients have a 75% increased risk of cancer compared to the general population, a meta-analysis confirms, leading the authors to recommend screening in these patients.Patients were at particular risk of developing (RR 4.35) and haematological...

» Morphea Scleroderma

Morphea, is one of two types of localized , and a disorder characterized by excessive deposits leading to thickening of the dermis, subcutaneous tissues, or both. The cause of Scleroderma is unknown. It is important to remember that it is not...

» Systemic Sclerosis: An Update

Systemic sclerosis (SSc) is a clinically heterogeneous, systemic disorder affecting connective tissue of skin, internal organs, and walls of blood vessels. It is characterized by alterations of the microvasculature in the form of hypoxia, digital...

» 12 Natural Symptom Relievers For Heartburn

Have frequent ? Take Nexium. An advertising blitz for that little purple pill helped it become the No. 2 best-selling prescription drug in the U.S., with sales of $6.3 billion last year. Now, researchers are warning that this category of heartburn...

Comments  

 
0 #2 adelwyn 2009-11-16 16:22
Exercise is always a good thing, and we shouldn't wait till we are not in good health to start.
Report to administrator
 
 
0 #1 trinischolar 2009-11-13 00:09
*low whistle* Love the info.! A lot of different medication though. Wish there is a listing of natural medications you can take for this disease. I strongly believe in the natural way.
It seems to me, though, that when taking these medications, you have to be exercising regularly. Correct me if I'm wrong. ;-)
Report to administrator