Scleroderma and Pulmonary Hypertension

Scleroderma is a connective tissue disease. Collagen is the strong fibrous stuff found in connective tissue. Connective tissue connects and supports parts of the body and is dense with blood vessels. While Scleroderma means "hard skin" this same hardening can happen to an organ or tissue because of rapid growth of too much collagen, which is my case. Most often death with Scleroderma is caused by pulmonary disease. About 150,000 to 300.000 in the U.S. have Scleroderma in the U.S., striking 4 times as many women as men. The cause of Scleroderma is unknown and there is not presently a cure. Scleroderma can lead to PH because it can cause scar tissue to build up in the lungs or heart. Roughly a third of systemic Scleroderma patients may develop mild PH. When mild, it can often go undetected for a number of years, not progressing. When symptoms are prevalent such as increased shortness of breath, it is much easier to diagnose and also much harder to treat. It is not known how often mild PH grows into serious PH as mine has. My pulmonary function tests have shown I have had mild PH in my lungs for several years. If a person has a diffusing capacity of less than 55 percent predicted, they probably have PAH. This is the ability of oxygen to pass from the air sacs of the lungs into the blood.
If a patient does not have a severe form of PH, doctors usually begin treatment by lowering lung pressures with calcium channel blockers, (Norvasc, Procardia, etc.), that come in pill form and are much lest expensive than alternative treatments. Unfortunately, few patients respond to this treatment.
If calcium channel blockers don’t work or a patient is in Class III, then endothelin receptor antagonists (traclear, etc.), some form of prostanoid theraphy (Flolan, Iloprost, ect.), and/or phosphodiesterase inhibitors (Viagra, etc,) will be considered. Letairis, which is what I just started is an endothelin receptor antagonist. Endothelin is present in the blood. Patients with PH triggered by other diseases tend to have higher-than-normal levels of it. It binds to receptors in the layer of smooth muscle cells that surround it. Once it binds. it causes vasoconstriction and retards the growth and development of new smooth muscle cells. It may also increase fibrous tissue formation. Designer molecules made in labs can keep endothelin from binding to receptors and are called endothelin receptor antagonists. Their great advantage is they come in pill form.
Before Flolan was available in 1995, class I or II PH patients lived an median of 6 years . Class III patients (of which I am one) lived 2.5 years and class IV patients lived only 6 months. Scleroderma patients with PH do not appear to live as long even with Flolan. When Scleroderma patients die it is usually from pulmonary disease, particularly pulmonary vascular disease. Letaris has shown that class III life expectancy can be increased to 5 years. I think this is with a patient whose PH is primary, not secondary such as me whose primary disease is Scleroderma.
My references regarding medical information come from the Pulmonary HYpertension Association, the Scleroderma Foundation, The Scleroderma Research Foundation, and The International Scleroderma Network. I am not a doctor, just a patient. Scleroderma affects each patient differently. What works for one patient may not work for another and may even be dangerous. I make an effort to provide reliable information. However, it is up to you to verify all facts and consult your own doctor before starting or discontinuing any treatments.