Interstitial lung disease (ILD) is a broad group of lung disorders that cause inflammation and scarring in the lungs. Sjögren’s disease, and other autoimmune diseases, can cause ILD.
What is interstitial lung disease?
When viewed by a high-resolution chest CT scan or under a microscope, ILDs can be divided into various subtypes. The most common ILD patterns in Sjögren’s include:
- Non-Specific Interstitial Pneumonia (NSIP): this is the most common type of ILD seen in patients with Sjögren’s and is characterized by both inflammation and scarring of the lung tissue.
- Lymphocytic Interstitial Pneumonitis (LIP): this pattern is seen more commonly in Sjögren’s as compared to any other autoimmune disease. LIP causes inflammation around the small airways and the surrounding lung tissue, often leading to the formation of air-filled holes (cysts) in the lungs.
- Organizing pneumonia (OP): this can cause multiple different types of abnormalities on chest imaging and generally responds well to treatment.
- Usual Interstitial Pneumonia (UIP): this type primarily causes scarring of the lung tissue and is generally considered the worst prognosis ILD subtype.
It’s estimated that 10 to 15 out of 100 people with Sjögren’s have ILD.
Symptoms of interstitial lung disease
ILD symptoms usually start out mild and worsen over time. In some cases, you might not notice any ILD symptoms at first. The most common symptoms of ILD include:
- Shortness of breath
- Dry cough
- Fatigue (feeling very tired all the time)
- Chest discomfort
You may have additional symptoms, depending on which form of ILD you have. Some ILD symptoms, like fatigue and a dry cough, are common symptoms of Sjögren’s disease, too, so pay close attention to your symptoms and discuss changes with your healthcare provider.
Because ILD doesn’t always cause symptoms right away, it’s important to ask your doctor to check your lungs regularly if you’ve been diagnosed with Sjögren’s disease. It is important to catch ILD as early as possible for the best treatment.
How are interstitial lung disease and Sjögren’s related?
Sjögren’s disease is a systemic disease that can damage any part of the body — including the lungs. Sjögren’s disease and other autoimmune diseases like lupus, scleroderma, and rheumatoid arthritis can all cause ILD.
How is interstitial lung disease diagnosed?
To diagnose ILD, your doctor will ask about your symptoms and use tests to examine your lungs and check how well they’re working. Tests that doctors use to diagnose ILD may include:
- Pulmonary (lung) function tests (PFTs), such as measuring how much air you inhale and exhale, and how much oxygen goes from your lungs to your blood.
- Imaging tests, such as an X-ray or CT scan, to check for damage to your lungs.
- Blood tests, to check for other conditions that are known to cause ILD.
- Bronchoscopy, which means inserting a thin tube with a camera on the end (called a bronchoscope) through the nose or mouth and into the lungs to check your airways and take samples from the lungs.
- Biopsy, which is when your doctor takes a small sample of tissue from your lungs and looks at it under a microscope to check for signs of ILD.
If you have Sjögren’s disease, it’s a good idea for your doctor to regularly check your lungs for signs of ILD or other lung problems.
If you’ve already been diagnosed with ILD and suspect you may also have Sjögren’s disease, talk with your doctor to get further testing for Sjögren’s disease.
What does having interstitial lung disease and Sjögren’s disease mean for me?
If you have ILD and Sjögren’s, it’s important to get treatment for both conditions and to know the signs of possible complications. Treating both conditions requires multidisciplinary care, usually including a rheumatologist (treating Sjögren’s) and pulmonologist (treating lung conditions like ILD).
The type of treatment you need for ILD depends on your symptoms, how much they bother you, and whether your symptoms get worse over time. Your doctor will monitor your condition with lung function tests and imaging to see if your ILD is stable or gets worse over time. Treatments may include:
- Taking medicines to help reduce inflammation and lung scarring
- Using supplemental oxygen, if your oxygen levels drop below a safe range
- Doing physical therapy or breathing exercises to help you breathe stronger and more easily
Having both Sjögren’s and ILD can make you more likely to experience certain complications. People with Sjögren’s ILD sometimes develop pockets of air within their lungs, called cysts. These cysts increase your risk of a serious problem called pneumothorax (when a lung collapses). It’s important to know the symptoms, which include:
- Shortness of breath that comes on very suddenly
- Chest pain that feels worse when you take a deep breath
Pneumothorax is an emergency and requires immediate treatment. If you have Sjögren’s disease and experience these symptoms, get help right away.
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