If you have systemic sclerosis (SSc), you’re at greater risk for lung problems. SSc causes collagen to build up in all your organs. When this happens in your lungs, it causes inflammation and scarring of the lung tissue, a condition called interstitial lung disease (ILD). Studies show that anywhere from one-third to one-half of people with systemic sclerosis develop this disorder.
If you notice symptoms like shortness of breath, low exercise tolerance, and a persistent cough, see your doctor right away. Early diagnosis and early use of preventative treatment is the key since once you have lung damage, it can’t be reversed. Your doctor may order one or several tests to find out if you have SSc-ILD.
Chest X-ray. This is often the first test given if you complain of shortness of breath and a nagging, persistent cough. If you have ILD, your doctor may see very fine white lines on your X-ray. This is from inflammation and swelling in your lungs that makes the tissue dense.
Computerized tomography (CT) scan. This imaging test is key to the diagnosis of interstitial lung disease. It combines X-rays with computer technology to get different images at different angles of your lungs. This allows your lungs and surrounding areas to be seen in greater detail. Make sure you get a high-resolution CT scan, which can detect even small abnormalities that could be ILD. It can also show exactly where lesions are, which can help guide treatment.
Echocardiogram. Systemic sclerosis can damage the blood vessels in your lungs. This lowers your body’s blood oxygen level. It also can cause a reflex increase in blood pressure in your pulmonary arteries, a condition known as pulmonary arterial hypertension (PH). An echocardiogram uses sound waves to visualize your heart to examine its structures and see whether or not it’s pumping normally, and to look for PH.
When you have systemic sclerosis, your body may make certain antibodies that increase your risk of having ILD. Your doctor may order blood tests to look for those. They might also order an arterial blood gas test, which measures the levels of both oxygen and carbon dioxide in your blood. This can show whether you are getting enough oxygen and your lungs are removing enough carbon dioxide.
Lung Function Tests
These tests measure your lungs’ ability to move air in and out. They include:
Spirometry. This test has you breathe out forcefully through a tube connected to a machine. It helps gauge how well your lungs hold and move air, as well as how easily oxygen can travel from your lungs into your bloodstream.
Lung diffusion test. You breathe in a very small amount of carbon monoxide through a mouthpiece, hold it for a few seconds, then breathe out. If the amount of carbon monoxide you breathe out is higher than normal, your lungs may have trouble with oxygen absorption. This can be a sign of ILD.
Oximetry. This simple test uses a small electronic device placed on one of your fingers to measure oxygen levels in your red blood cells. You can do it while sitting or as part of a 6-minute walk test. If your oxygen levels drop with activity, it may signify ILD.
Most people with systemic sclerosis can be diagnosed with ILD through a combination of imaging and lung function tests. In certain cases, your doctor may want to examine a small amount of your lung cells under a microscope. This is called a biopsy. It can be done in a few ways:
Trans-bronchial biopsy. Your doctor passes a thin tube through your mouth or nose to look inside your lungs and remove a small piece of lung tissue. Checking your lungs with this tube is called a bronchoscopy. It can be done either under sedation or general anesthesia. It’s very safe, although you might have some minor bleeding a temporary sore throat, hoarseness, and a cough afterward.
Bronchoalveolar lavage. This is when your doctor inserts saline solution through a tube to wash your airway, then removes it. The fluid that comes out contains cells and other things like bacteria from your lungs’ air sacs. The doctor will test this in the lab.
Surgical biopsy. Lung biopsies are more invasive and can have more complications than other procedures. But you may get one if they don’t provide a clear diagnosis. They’re usually done under general anesthesia laparoscopically, which means the surgeon views your lungs and takes tissue samples via a small video monitor that’s inserted between your ribs.