PH and Scleroderma

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I was just diagnosed with scleroderma, which I don’t fully understand. My doctor has asked me if I get short of breath or feel fatigued or like I’m going to faint. Why am I being asked this?

Scleroderma, which means “hard skin” in Latin, is a type of autoimmune disease that affects the connective tissues and blood vessels of the body. Connective tissue is mostly made of collagen and gives structure and support to the organs of the body. Collagen is a protein that the body uses to make scar tissue to repair itself. In scleroderma, the immune system attacks the body’s organs and tissues. As a result, excess collagen is produced and scar tissue forms.

The additional symptoms your doctor is mentioning (shortness of breath and fatigue) are those of pulmonary arterial hypertension (PAH), a disease of the small arteries of the lungs. PAH is a common complication of scleroderma. Between eight to 12 percent of all scleroderma patients develop PAH.

In patients suffering from PAH, arteries become damaged and narrowed, making it difficult for the right side of the heart to pump blood through the lungs. Your doctor, therefore, wants to make sure right away that you’re not developing PAH.

Scleroderma patients tend to have exercise limitations and it is important to report any changes in your current abilities. Unexplained shortness of breath or increasing fatigue, swelling of the ankles, legs, abdomen or arms, chest discomfort or pain, and light-headedness and fainting are all symptoms your doctor will want to know about, since these can also be signs of pulmonary arterial hypertension.

What can happen to patients with these problems, and why should I be concerned?

It is absolutely essential that you as a scleroderma patient be aware of your condition, and that you stay in touch with your doctor concerning your symptoms. While many scleroderma patients go on to lead long and enjoyable lives, PAH is a serious and complex condition that requires immediate treatment. Pulmonary complications are the leading cause of death in people with scleroderma.

In general, the medications that are used to treat regular PAH do not work as well when used on patients who suffer from PAH and scleroderma together. A possible explanation of this is that the combination of PAH and scleroderma occurs in older patients who might already have other forms of lung disease.

It is also possible that patients with scleroderma have more a severe form of PAH of their pulmonary arterioles or that the right side of the heart is less able to handle the higher pressures caused by PAH. In all cases, the earlier a diagnosis is made, the sooner treatment can begin, and the better the outlook is for the patient.

What tests can be done?

Since patients with scleroderma are typically under a doctor’s care for their scleroderma, annual screening for PAH and PH should be done. Screening should include a detailed history about the level of physical activity and if any problems are limiting those activities. The symptoms mentioned before (shortness of breath, swelling, etc.) should always be brought to a doctor’s attention.

A pulmonary function test can help check for changes in the lungs associated with scleroderma. Echocardiography is a useful screening tool although it may not always be accurate if a patient is in the earlier or milder stages of PH. A blood test called serum BNP (brain natriuretic peptide assay) is useful as well, and can help doctors identify several problems. Right heart catheterization remains the most accurate diagnostic test and provides other information about the heart’s condition as well.

If I have PAH as well as scleroderma, what can doctors do to help me?

All patients benefit from simple basic measures including attention to fluid balance and therapies that improve the efficiency of the heart. These may include supplemental oxygen or the medication digoxin, as well as anticoagulants (blood thinners).

Once a doctor and patient have been able to establish an effective medical approach, many patients show significant improvement, often in conjunction with doctor-prescribed cardiopulmonary rehabilitation training (CPRT).

Through the years, a variety of medications have been shown to slow the damage and relieve the symptoms caused by PAH. These medications are very complex, and choosing the correct initial treatment requires the knowledge and expertise of a physician who specializes in PAH. Professionals at pulmonary hypertension centers can help identify pulmonary hypertension and choose the correct type of treatment.

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