Have you ever felt short of breath for no real reason? For some living with lupus, this could be a sign of a rare but serious complication—shrinking lung syndrome (SLS). Read on to understand the symptoms, causes, and treatment options that just might help you breathe a little easier!

• Introduction
• The Biology of Shrinking Lung Syndrome
• Symptoms and Diagnosis
• Treatments and Medications
• Living with Shrinking Lung Syndrome
• In Conclusion

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Introduction

While lung complications in lupus are not uncommon, one lesser-known but particularly concerning condition is Shrinking Lung Syndrome (SLS).  This rare complication was first described in 1965 and can cause breathing difficulties, chest pain, and decreased lung function.  Although it sounds alarming, the good news is that with the right diagnosis and treatment, many people with SLS can manage their symptoms effectively.

SLS is estimated to occur in between 0.5% to 1.5% of all lupus patients with an average age at diagnosis of 36 years. It is also reported, though less common, in those with Sjogren’s disease, rheumatoid arthritis, and scleroderma.

In this article, we’ll break down what SLS is, why it happens, how it’s diagnosed, and what treatments are available. Whether you have just heard of SLS for the first time or you have been experiencing unexplained breathing issues, this article will help you understand what is going on and how to take action.

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The Biology Behind Shrinking Lung Syndrome

SLS is characterized by a progressive reduction in lung volume, making it difficult for the lungs to expand fully. Unlike other lung conditions, SLS is not caused by fibrosis (scarring) or inflammation directly damaging lung tissue. Instead, it is believed to be due to problems with the diaphragm—the large muscle that controls breathing.

In people with SLS, the diaphragm may become weakened or have reduced movement, limiting the lungs’ ability to expand. Some researchers believe that inflammation or nerve involvement in lupus could contribute to this problem. Others suggest that pain caused by lupus-related pleurisy (inflammation of the lung lining) leads to shallower breathing over time, weakening the respiratory muscles. Regardless of the exact cause, the end result is difficulty in taking deep breaths and reduced lung function.

Symptoms and Diagnosis

It is rarely the first symptom of lupus, and it is unpredictable – occurring anytime from the first 4 months up to 24 years after diagnosis.  Symptoms can be subtle at first, but tends to worsen over time, and it is critically important to diagnose early.

Common signs include:

• Shortness of breath (dyspnea): Especially noticeable during physical activity.
• Chest pain: Often sharp and worse when breathing deeply.
• Shallow breathing: Feeling like you can’t take a full breath.
• Fatigue: Due to decreased oxygen intake.
• Reduced exercise tolerance: Even mild exertion can cause breathlessness.

Diagnosing SLS can be tricky because its symptoms can mimic other lung or heart conditions common in lupus, such as pleurisy, pulmonary hypertension, or interstitial lung disease. A doctor may use the following tests to confirm the diagnosis:

• Pulmonary function tests (PFTs): These measure lung volume and capacity. People with SLS often show a restrictive pattern, meaning their lungs can’t expand properly.
• Chest X-ray or CT scan:  While SLS doesn’t cause visible scarring, imaging can help rule out other lung diseases. The most common feature is an unusual raising or elevation of the diaphragm in chest x-rays, either of one lung or both.
• Diaphragm ultrasound or fluoroscopy: These tests check the movement of the diaphragm to see if it is functioning properly.

 

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Treatment Options and Medications

The good news? SLS is treatable! While there’s no single cure, a combination of medications and physical therapy can improve breathing and lung function.

Corticosteroids and Immunosuppressants

Since SLS is often linked to lupus-related inflammation, medications that control lupus activity can help. Doctors may prescribe:

• Prednisone: A corticosteroid that reduces inflammation and may help improve lung function.
• Methotrexate, azathioprine, or mycophenolate mofetil: Immunosuppressants that help control lupus and prevent further complications.

Bronchodilators and Respiratory Therapy

• Bronchodilators: While typically used for asthma, some doctors prescribe them to help open airways and make breathing easier.
• Pulmonary rehabilitation: This is a fancy way of describing breathing exercises and physical therapy, which can strengthen the diaphragm and improve lung capacity.

Pain Management

Since chest pain and pleurisy can contribute to shallow breathing, so managing pain is important. This can include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs): Like ibuprofen to reduce pain and inflammation.
• Physical therapy and breathing exercises: Techniques such as diaphragmatic breathing can retrain the lungs to expand fully.

Oxygen Therapy (In Severe Cases)

For people with significantly impaired lung function, supplemental oxygen may be necessary to improve oxygen levels in the blood.

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Living with Shrinking Lung Syndrome

A diagnosis of SLS can feel overwhelming, but many people with lupus successfully manage this condition with the right approach. Here are some tips for daily life:

• Stay active: Light exercise, like walking or yoga, can keep your lungs strong.
• Practice deep breathing exercises: These can help improve lung expansion and diaphragm strength.
• Follow your treatment plan: Taking medications as prescribed can prevent worsening symptoms.
• Communicate with your doctor: Report any changes in symptoms to ensure you get the best care.

In Conclusion

Shrinking lung syndrome is a rare but serious complication of lupus that affects lung function and breathing. While it can be difficult to diagnose, understanding the symptoms and working with your healthcare team can make a significant difference. Treatments like corticosteroids, breathing exercises, and pain management strategies can help improve lung capacity and quality of life.

If you’re experiencing unexplained shortness of breath or chest pain, don’t ignore it—talk to your doctor. With the right care, you can continue living a full and active life despite SLS. Remember, knowledge is power, and taking charge of your health is the first step toward breathing easier.

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References

Borrell, H., Narváez, J., Alegre, J.J., Castellví, I., Mitjavila, F., Aparicio, M., Armengol, E.,  Molina-Molina, M., & Nolla, J. (2016). Shrinking lung syndrome in systemic lupus erythematosus: A case series and review of the literature. Medicine 95(33), e4626, https://journals.lww.com/md-journal/fulltext/2016/08160/shrinking_lung_syndrome_in_systemic_lupus.53.aspx

Choudhury S, Ramos M, Anjum H, Ali M, and Surani S. (2020). Shrinking lung syndrome: A rare manifestation of systemic lupus erythematosus. Cureus, 12(5) e8218. https://pmc.ncbi.nlm.nih.gov/articles/PMC7306665/

Colquhoun, M., & Akram, S. (2023). Shrinking lung syndrome. StatPearls.https://www.ncbi.nlm.nih.gov/books/NBK568683/

 Fosam, H. (2017, September 11). Advancing Understanding of Shrinking Lung Syndrome in SLE. Rheumatology Advisor. https://www.rheumatologyadvisor.com/features/advancing-understanding-of-shrinking-lung-syndrome-in-sle/

Parmar, M., Bajwa, A., Hussain, S.T., & Bhwardwah, H. (2022). Shrinking lung syndrome: A rare manifestation of systemic lupus erythematosus. CHEST Journal, 162(4). https://journal.chestnet.org/article/S0012-3692(22)03152-X/fulltext

Warrington, K.J., Moder, K.G., & Brutinel, W.M. (2000). The shrinking lungs syndrome in systemic lupus erythematosus. Mayo Clinic Proceedings, 75(5). 467-472. https://www.mayoclinicproceedings.org/article/S0025-6196(11)64215-8/fulltext

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Author: Greg Dardis, MS      

Professor Dardis was formerly the Chair of the Science Department at Marylhurst University and is currently an Assistant Professor at Portland State University and the American College of Healthcare Sciences.  His focus has been human biology and physiology with an interest in  autoimmunity.  Professor Dardis is also a former President of the Board of Directors of Kaleidoscope Fighting Lupus.

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