Symptoms of lung disease may include cough and shortness of breath, particularly when climbing stairs and walking uphill.
However, these symptoms do not necessarily imply the presence of lung disease, as a cough can have other causes, such as chronic heartburn, and breathlessness on exertion can be related to joint and muscle pains.
On the other hand, some individuals can be so limited by joint or muscle problems that they do not exert themselves to a degree that causes breathlessness even when they have lung involvement. Furthermore, when the lung disease is limited in extent, it may not be associated with any symptoms.
Therefore, tests are needed to accurately assess the presence and extent of lung disease. These include lung function (breathing) tests, which allow an evaluation of how well the lungs work, and a CT (computed tomography) scan of the chest, which picks up even mild degrees of lung involvement. Together, the lung function tests and the CT scan are used to estimate whether lung fibrosis, if present, is mild or extensive.
As part of the initial assessment for patients diagnosed with scleroderma, an echocardiogram, an ultrasound scan which looks at the heart, is usually also performed. The echocardiogram is used to rule out any significant problem with the heart and to determine if there are any signs of pulmonary hypertension.
In some individuals, a bronchoscopy will be considered. Bronchoscopy involves passing a flexible fibre-optic camera into the airways in order to obtain samples of the inflammatory cells within the lungs. Not everyone with scleroderma needs a bronchoscopy; however, if your physician feels that it could be useful, they will discuss bronchoscopy with you in more detail. One of the reasons to perform a bronchoscopy is to check for possible infection.
Scleroderma (or systemic sclerosis) is a chronic disease associated with skin thickening and changes to blood vessels, particularly those supplying the fingers and toes.
Meet the ILD team
Consultant physicians
Professor Athol Wells (clinical and academic lead for ILD)
Dr Toby Maher
Dr Elizabeth Renzoni
Dr Felix Chua
Dr Peter George
Dr Philip Molyneaux
Clinical nurse specialist
Research nurse
Anne-Marie Russell
Interstitial lung disease unit
Lind ward, Fulham wing/South block, Royal Brompton Hospital
Telephone: +44(0)20 7351 8327
Fax: +44(0)20 7351 8918