Interstitial lung involvement and Sjögren’s

Sjögren’s is often associated with dry eyes and dry mouth, but for some people the disease affects far more than the glands. One of the most serious and under-recognised complications is interstitial lung involvement.

Lung involvement in Sjögren’s is not rare, yet it is frequently missed, diagnosed late, or dismissed as unrelated to the autoimmune process. For patients, this can mean delayed treatment, worsening symptoms, and unnecessary progression of lung disease.

Understanding how Sjögren’s can affect the lungs is critical for early detection and appropriate care.

What is interstitial lung involvement

The interstitium is the network of tissue that supports the air sacs in the lungs. When this tissue becomes inflamed or scarred, it can interfere with oxygen exchange. This group of conditions is broadly referred to as interstitial lung disease.

In Sjögren’s, interstitial lung involvement is caused by autoimmune inflammation. Over time, this inflammation can lead to fibrosis, which is permanent scarring of lung tissue.

Not everyone with Sjögren’s will develop lung involvement, but for those who do, early recognition can significantly affect outcomes.

How Sjögren’s affects the lungs

Sjögren’s is a systemic autoimmune disease, meaning it can affect multiple organs beyond the glands. In the lungs, immune-mediated inflammation can involve:

• The interstitium, leading to interstitial lung involvement
• The airways, causing chronic cough or bronchial disease
• The pleura, the lining around the lungs
• Pulmonary blood vessels in rare cases

Interstitial lung involvement is one of the more serious manifestations because it may progress silently before symptoms become obvious.

Common symptoms to watch for

Symptoms of lung involvement in Sjögren’s can be subtle and are often mistaken for other conditions. These may include:

• Persistent dry cough
• Shortness of breath, especially with exertion
• Chest tightness or discomfort
• Reduced exercise tolerance
• Unexplained fatigue that worsens with activity

Because fatigue and breathlessness are common in many conditions, lung involvement may be overlooked or attributed to deconditioning, anxiety, asthma, or ageing.

Why interstitial lung involvement is often missed

There are several reasons lung involvement in Sjögren’s is frequently underdiagnosed:

• Symptoms may develop gradually, making changes easy to dismiss
• Chest X-rays can appear normal in early disease
• Standard lung function tests may not detect subtle interstitial changes
• Awareness of Sjögren’s-related lung involvement remains limited among non-specialists

In some cases, lung disease is identified before Sjögren’s itself is diagnosed, leading to fragmented care and delayed recognition of the underlying autoimmune cause.

Diagnosis and monitoring

Diagnosing interstitial lung involvement usually requires a combination of clinical assessment and imaging. This may include:

• High-resolution CT scans of the chest
• Pulmonary function tests, including diffusion capacity
• Autoimmune blood tests
• Multidisciplinary assessment involving rheumatology and respiratory specialists

Once identified, ongoing monitoring is important. Lung involvement can remain stable, progress slowly, or worsen more rapidly depending on the individual and the pattern of disease.

Treatment and management

There is no single treatment approach that suits everyone. Management depends on the severity, progression, and specific pattern of lung involvement.

Treatment may include immunosuppressive or immunomodulatory medications to reduce inflammation. In some cases, antifibrotic therapies may be considered. Supportive care such as pulmonary rehabilitation, vaccination, and infection prevention also plays an important role.

Early intervention offers the best chance to slow progression and preserve lung function.

The patient experience

For many people with Sjögren’s, lung involvement adds another layer of uncertainty and fear. Breathlessness can limit daily activities, impact independence, and significantly affect quality of life.

Patients often report feeling dismissed when early symptoms are raised, especially if imaging or basic tests appear normal. This can lead to delays in diagnosis and treatment, reinforcing feelings of being unheard or underestimated.

Listening to patient concerns and recognising subtle changes is essential.

Why awareness matters

Greater awareness of interstitial lung involvement in Sjögren’s benefits both patients and clinicians. Early recognition allows for timely referral, appropriate investigations, and informed monitoring.

For patients, understanding that lung symptoms can be part of Sjögren’s helps validate experiences and supports self-advocacy in clinical settings.

Sjögren’s is not just a dryness condition. Its systemic nature means vigilance is required to protect organs such as the lungs.

Interstitial lung involvement is a serious but often overlooked aspect of Sjögren’s. While not everyone will be affected, those who are deserve early recognition, accurate information, and coordinated care.