When you think of Sjögren’s disease, you’re more likely to think of common symptoms like dry eyes and a dry mouth than anything related to breathing. But, like other autoimmune diseases, Sjögren’s has a way of affecting unexpected parts of your body. This includes your lungs and respiratory system.

If you have Sjögren’s disease, a persistent cough might be more than just a long-lasting cold. Here, we break down all of the ways Sjögren’s can affect your lungs and how to manage Sjögren’s respiratory complications.

How Sjögren’s Disease Can Affect the Lungs

Sjögren’s disease is widely recognized as a condition affecting the eyes and mouth. More broadly, Sjögren’s is an autoimmune disease that causes dryness in any moisture-producing area of the body. An overactive immune system damages glands that produce moisture in your eyes, nose, throat, skin, and other places.

For some people, Sjögren’s causes inflammation in and around the alveoli (small air sacs in the lungs). The alveoli are responsible for exchanging oxygen and carbon dioxide. Oxygen goes into your blood from your lungs, and carbon dioxide is removed. Inflammation can disrupt this process and damage other parts of the lungs, too.

When your nasal passages are constantly dry, as they often are with Sjögren’s, you’re also at risk for frequent sinus infections.

Signs of Lung Problems With Sjögren’s

Lung damage from Sjögren’s disease can cause several symptoms that indicate lung disease. Other Sjögren’s symptoms can impact breathing because of the way Sjögren’s dries out the nose and airway. You might experience:

• Dry nose
• Frequent nosebleeds
• Postnasal drip
• Hoarse voice
• Chronic (long-lasting) dry cough
• Shortness of breath
• Chest pain or tightness

The specific symptoms can help your doctor determine which part of your respiratory system is affected and if you have any lung complications. Certain Sjögren’s lung complications can cause symptoms that appear unrelated to the lungs, such as leg swelling.

Main Types of Lung Problems Related to Sjögren’s

Multiple lung and respiratory diseases can arise from Sjögren’s dryness or inflammation. Sjögren’s disease can affect anywhere from the nose and upper airway down to the lungs. Here are a few of the most common lung complications.

Interstitial Lung Disease

Interstitial lung disease, also called interstitial pneumonitis, involves inflammation that damages and thickens the lining around the alveoli, called the interstitium. It affects 10 percent to 15 percent of people living with Sjögren’s disease.

When the interstitium is thick and damaged, your lungs have trouble delivering oxygen to your blood and removing carbon dioxide. You may have trouble breathing as a result.

Pulmonary Hypertension

Pulmonary hypertension (PH) is a type of high blood pressure that occurs in the lungs. It happens when the blood vessels in your lungs become narrow and stiff, which can be the result of Sjögren’s disease-related damage to the lung tissue. PH can also occur as a reaction to low blood oxygen levels after inflammation damages the alveoli.

Shortness of breath, chest pain, coughing, and leg swelling can all be signs of PH. If these symptoms appear suddenly, you might have a pulmonary embolism (blood clot in the lung). Pulmonary embolism is a medical emergency, so you should get medical attention right away if you notice sudden and severe symptoms.

Follicular Bronchiolitis

Follicular bronchiolitis (FB) is one of the most common lung complications associated with Sjögren’s disease. Outside of Sjögren’s and other connective tissue diseases like rheumatoid arthritis, FB is quite rare and often misdiagnosed.

FB originates in the small airways and can cause a persistent cough and trouble breathing during exercise. You might also experience frequent bronchitis (small airway inflammation) and sinus infections.

Chronic Obstructive Pulmonary Disease

Chronic obstructive pulmonary disease (COPD), a condition most commonly seen in people who smoke, can also be a lung complication of Sjögren’s disease. In a Swedish study that examined people with primary Sjögren’s disease over 11 years, 37 percent of participants eventually met the criteria for COPD.

COPD includes chronic and irreversible lung damage that restricts airflow and makes it harder to breathe. As with other Sjögren’s-related lung diseases, this damage often comes from inflammation or scarring in the lungs that narrows your airways. COPD symptoms, such as a productive cough and trouble taking a deep breath, can flare up and get worse at times. These flare-ups are called exacerbations, and they can be severe enough to require hospitalization.

COPD progressively worsens over time, so it’s important to work closely with a pulmonologist to manage it. You might experience frequent complications like pneumonia, hypoxemia (low oxygen levels), or a collapsed lung.

How Lung Problems Are Diagnosed

Not everyone with Sjögren’s-related lung disease will experience symptoms. In a large study of people with Sjögren’s disease, pulmonary function tests (PFTs) and imaging tests revealed that up to 75 percent had lung abnormalities of some kind, even if they didn’t have symptoms.

Whether or not you have symptoms like a chronic cough or shortness of breath, your doctor may use certain tests to learn more about how Sjögren’s disease affects your lungs.

Pulmonary Function Tests

PFTs can tell you how well your lungs are working by measuring the amount of air that goes in and out of them. There are multiple types your doctor can use when evaluating lung damage:

• Spirometry — Measures how much air you can inhale, exhale, or hold in your lungs
• Cardiopulmonary exercise test — Measures the function of your lungs and heart during exercise
• Gas diffusion study — Measures how much oxygen goes from your lungs into your blood

Imaging Tests

Imaging tests like chest X-rays and CT scans allow your doctor or pulmonologist to examine your lungs and identify visible signs of inflammation or scarring. If you have interstitial lung disease, a chest CT scan can tell you which type you have. Rarely, you might need a lung biopsy after imaging testing.

Echocardiogram

An echocardiogram is a test that evaluates your heart function. Doctors use it to screen for PH and heart complications when you have shortness of breath.

Protecting Your Lung Function

Your doctor can recommend treatment for Sjögren’s-related lung disease if you’ve been diagnosed with interstitial lung disease, COPD, or other complications. They may recommend regular PFTs to track your lung function over time, even if you have few or no symptoms.

Managing Sjögren’s disease with immunosuppressive therapy or corticosteroids might help improve lung diseases or prevent further damage to your lungs. If you have symptoms like a persistent cough or dry nose, you can ease symptoms and protect your lung function by taking the following steps:

• Drink plenty of fluids.
• Use a humidifier — Humidifiers moisturize the air so you can breathe easier and experience fewer nosebleeds.
• Clear your sinuses — A sinus rinse or neti pot can clear debris from your airways and nasal passages.

Treatments like oxygen therapy or pulmonary rehabilitation might be necessary to preserve your lung function if you have COPD, PH, or other lung diseases related to Sjögren’s disease.

When To Contact Your Doctor

Certain lung diseases associated with Sjögren’s disease can get worse over time or cause serious complications like pneumonia or cysts. If you have symptoms like shortness of breath or a persistent cough that won’t go away, be sure to tell your doctor.

Certain lung complications are medical emergencies. Seek immediate medical care if you notice:

• Sudden, unexplained shortness of breath
• Chest pain that gets worse with deep breaths
• Bloody mucus when you cough
• Feeling like you’re going to pass out

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