Lung Involvement In Scleroderma

Background

Just as scleroderma could affect any organ by limiting its functions, the function of the lungs is also greatly interfered with if a patient would get scleroderma in his or her lungs. This is seen by numerous lung illnesses such as shortness of breath and coughing. This in turn can also cause problems with the heart such as hypertension and other heart illnesses.

Scleroderma usually begins with Raynaud’s phenomenon which happens on the skin. If the case is limited scleroderma, it would stop there however if it develops into something worse and would start affecting organs within, then that condition is known as systemic sclerosis. This is more likely to happen in women than it is on men and it usually develops somewhere between the ages of 30 and 50. Scleroderma rarely occurs in children.

How Are The Lungs Involved In Scleroderma?

Scleroderma happens when the antibodies in a person’s immune system would attack their own tissues, which is contradicting to their main purpose of protecting it. The cause of this happening is not yet known although some theories have been made. Some have said that the cause of it is genetic and that scleroderma is hereditary while others would say that scleroderma is brought about unwanted factors in the environment such as bacteria or viruses.

If scleroderma reaches the lungs, then that is the beginning of pulmonary involvement in scleroderma. The first signs that can be seen are shortness of breath and dry cough without mucus. If this worsens, this would lead to lung problems that are much worse and even hypertension. Fortunately, medications and other treatments are available for cases of pulmonary involvement in scleroderma.

What Are The Types Of Lung Involvement In Scleroderma?

It is said that about 10% to 15% of all people with lung involvement in scleroderma would develop sever lung disease during the duration of their illness. There are two main manifestations of lung involvement in scleroderma, one is interstitial lung disease. This is also known as fibrosing alveolitis or pulmonary fibrosis. This occurs in about 75% of all people with scleroderma.

Another manifestation is pulmonary vascular disease, which is the lung problem that could lead to hypertension. This occurs more frequently as another pulmonary complication, but it can also be a result of pulmonary fibrosis. This could happen from 10% to about 80% of all cases of scleroderma. Other manifestations of lung involvement in scleroderma would include brochiectasis, aspiration pneumonia, neoplasm, spontaneous pneumothorax and drug-associated pneumonitis.

How Can This Be Treated Or Managed?

There has been no known cure for lung involvement in scleroderma and as well as all other cases of scleroderma, it can however still be treated with a different approach. Instead of attempting to completely remove scleroderma from a patient, the typical approach for treating lung involvement in scleroderma is to prevent further damage or to attempt to restore normal lung activity with the use of medications, therapy or surgery for some cases.

The most used drug in treating pulmonary scleroderma are corticosteroids. These are also work more effectively when taken with cyclophosphanide. However, not all cases have been effectively treated with it. If ever a patient should develop side effects or if it would lead to further complications, the patient should consider other medications such as HRCT, BAL or penicillamine.