The lungs may be affected in scleroderma in two ways. First there may be inflammation within the lungs (termed alveolitis) which may eventually lead to scarring or fibrosis. Secondly, problems can develop with the blood flow through the lungs. There have been major advances over recent years related to the testing for and treatment of lung problems in scleroderma. Because these problems generally develop after the disease is diagnosed, it is important that patients are assessed properly and regularly. Only a minority of patients will develop problems of sufficient severity to require treatment but it is important that these cases are not missed, particularly because both circulatory and fibrotic problems are probably most treatable at the early stages.
Tests which are performed regularly include routine chest X-rays, lung function tests, ECGs and echocardiograms. More sophisticated investigations, including various forms of lung scan (e.g. CT scan or DTPA scan) are also used in some specialised centres, and sometimes more extensive procedures such as bronchoalveolar lavage to look for inflammatory cells or an operation to remove a piece of lung for examination under the microscope (a lung biopsy). A right heart catheter to examine the heart and the circulation in the lungs may also be required for some patients.
Treatment for lung problems will generally only be administered when the process appears to be active or progressive. For this reason it is important to repeat some of the tests listed above at intervals during the illness. This provides a screen for new problems and an assessment of established ones. It is important to view these regular tests as a positive event in that they allow your doctors to detect problems early and so consider treatment. Treatments include drugs to inhibit inflammation within the lungs and also to open up blood vessels. Although the lung is often involved in scleroderma not everyone will have symptoms suggestive of lung disease. It is important to identity lung disease at the earliest stage so that treatment can be commenced to attempt to prevent disease progression, as occasionally the severity of the disease is such, that patients become breathless on slight exertion and even at rest. Regular lung assessment is advisable. Studies of lung disease of either type in systemic sclerosis have suggested that the earlier the disease is detected, the more likely there is to be a good response to treatment. Lung disease in scleroderma usually has a long slow progression and patients have to learn to adjust to what they can and can’t do. It is advisable to do regular exercises and try to maintain a good basic level of physical fitness.
To download our leaflet on the Lung in Scleroderma click here.
For information on Pulmonary Hypertension (PAH) click here.
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