Co-Authored By: Dr. Akshay Muralidhar
Scleroderma is part of a special group of diseases called connective tissue diseases. This group of diseases includes lupus, rheumatoid arthritis, mixed connective tissue disease and many others. A key feature of these diseases is that the disease may involve multiple parts of their body including skin, joints, lung, heart, kidney, blood vessels and the gastrointestinal system. Some patients may just have joint pain while others may have symptoms of shortness of breath, chest pain, and gastrointestinal symptoms.
We have made great progress in treating certain aspects of scleroderma. In the past, kidney failure was a major problem for patients. With modern treatments, we rarely encounter kidney failure. Unfortunately, pulmonary arterial hypertension remains an important problem. In fact, a key part of the routine care of scleroderma patients is annual testing looking for the development of pulmonary arterial hypertension. Don’t wait for symptoms to develop to look for pulmonary arterial hypertension.
Annual screening in scleroderma patients includes an echocardiogram (heart ultrasound), lung function tests and an office visit with a doctor experienced in the care of scleroderma patients. Attention to gastro-esophageal reflux (GERD) is also very important. GERD is an important cause of lung disease and can be prevented by avoiding late meats and sleeping with the head of your bed elevated.
If your echocardiogram suggests that the pulmonary artery pressure is even mildly elevated, all major guidelines encourage an evaluation by a pulmonary hypertension specialist. In my practice, if a patient with scleroderma has an echocardiogram that suggests even mildly elevated pulmonary artery pressure then we proceed with right heart catheterization (the gold standard test to directly measure the pressure in your pulmonary arteries).
Right Heart Catheterization
Just because your echocardiogram suggests pulmonary hypertension, this does not mean that you have pulmonary arterial hypertension. The echocardiogram is an imprecise tool and often over-estimates the actual pressure. Additionally, the echocardiogram is not able to separate diastolic heart failure from pulmonary arterial hypertension. This requires a right heart catheterization. We often find that patients have normal pressures when we perform right heart catheterization or we discover that they have mildly increased pressure on the left side of the heart. In patients confirmed to have pulmonary arterial hypertension by right heart catheterization, we have many different treatments that improve symptoms of shortness of breath and improve survival. Although we don’t have a medication that cures scleroderma yet, we have very effective treatments for pulmonary arterial hypertension.