Lupus and Serositis
Lupus has a way of showing up in unexpected places, and sometimes that includes the protective linings around the lungs, heart, or abdomen. This condition, called serositis, may not be as well-known as other lupus symptoms, but it is surprisingly common and it can lead to serious problems. Read on to better understand serositis and learn what you can do about it.
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Introduction and Quick Facts
Systemic lupus erythematosus (SLE), or lupus, is a chronic autoimmune disease that can affect nearly every organ system. One of its important but less frequently discussed complications is serositis. “Serositis” means inflammation in one or more of your serous membranes – specifically the thin membranes that surround the heart, lungs, and line the abdominal cavity.
Some quick facts:
- Studies show that between 12% and 60% of lupus patients will experience some form of serositis during their illness – that is a big range!
- The American College of Rheumatology lists serositis as one of the key diagnostic criteria for lupus, highlighting its clinical significance (Petri et al., 2012).
- Typical symptoms include sharp chest pain (worse with deep breathing or lying down), shortness of breath, or abdominal pain and bloating.
- Because the effectsof serositis can mimic other serious conditions like heart attacks or infections, it can be difficult to diagnose.
- Is serositis dangerous? Most cases are treatable, but untreated inflammation can lead to very serious complications such as fluid buildup around the heart or lungs. So, a quick diagnosis and treatment are essential.
- Most cases respond to anti-inflammatory drugs, corticosteroids, or standard lupus medications like hydroxychloroquine. Severe or resistant cases may require immunosuppressants or biologics.
In the following sections, we’ll take a closer look at what serositis is, how it develops in lupus, and the steps patients and doctors can take to manage it effectively.
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Lupus and Serositis – the Details!
As mentioned, serositis refers to inflammation of the body’s serous membranes – the thin tissues that surround your internal organs. These membranes also produce fluid that reduce friction between each organ as it moves – such as the heart, lungs and all the organs in the abdominal cavity.
Pericardium: Around the heart is the double-layered pericardium or pericardial sac that not only helps protect the heart, but maintains the smooth, lubricated surfaces necessary for the heart to move as effortlessly as possible with each strong beat.
Pleura: Surrounding each lung are two layers of pleural membranes that allow the lungs to expand and contract with almost no friction against the ribs. They also ensure that the lungs remain air tight!
Peritoneum: These protective membranes line the abdominal cavity and surround many internal organs, like your intestines, liver, and others. Interestingly, this does not include the kidneys which are anatomically separate from the rest.
Each of these linings can also harbor cells of the immune system, like white blood cells, as a defense against infection – and this can be an issue when it comes to autoimmunity.
In lupus, the immune system can attack and irritate these tissues, leading to pain and swelling or effusion, the accumulation of fluid inside the membranes. Serositis is often a sign of active lupus disease and can be the consequence of a lupus flare, or triggered by an infection, or even stress.
Unlike rashes or other external signs of lupus, inflammation of serous membranes cannot be seen – though it can definitely be felt! The most important types include:
- Pericarditis: Inflammation of the sac around the heart, producing chest pain that worsens when breathing deeply or lying down, but improves when sitting up. Some form of pericarditis affects up to 25% of lupus patients.
Fluid can also build up in the pericardial sac – a condition called, pericardial effusion. It is a relatively common result of pericarditis and in some patients, it is the first sign of lupus!
- Pleuritis (pleurisy): Inflammation around the lungs, often causing sharp, chest pain and shortness of breath especially when lying down. Pleuritis is the most common, affecting 20%–60% of those with lupus.
Pleural effusion or fluid accumulation around the lungs is also fairly common.
- Peritonitis: Less common in lupus, this affects the abdominal lining and can cause tenderness, bloating, and discomfort. Peritonitis is more rare, but can appear if the lupus is severe enough.
Because chest pain or abdominal pain can also signal other emergency conditions like heart attacks or infections, it is important to get these symptoms checked out by your healthcare provider quickly!
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Diagnosis
A diagnosis of lupus-related serositis, like lupus itself, may involve several steps:
- Clinical evaluation: Identifying chest or abdominal pain patterns, and listening for “friction rubs.”
- Laboratory testing: Checking for increased lupus activity with ANA, anti-dsDNA, complement level tests.
- Imaging: Chest X-rays for pleural effusion, echocardiogram for the buildup of pericardial fluid, and CT scans or ultrasounds for peritoneal inflammation.
The challenge is ruling out other causes like pneumonia, pulmonary embolism, or cardiac ischemia, which may look similar but require different treatment.
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Treatment and Management
Note: Always talk to your healthcare provider before making ANY changes to your treatment plans.
Treatments are generally similar to those for lupus flares and depend on severity:
- Mild cases: Often, NSAIDs can relieve much of the pain and inflammation.
- Moderate to severe cases: Corticosteroids (like prednisone) are often prescribed.
- Long-term Management: Hydroxychloroquine is standard for all patients with lupus and is helpful in managing serositis.
- Refractory or Difficult Cases: Changes to your current lupus treatment plan may be necessary, and may include the usual suspects: immunosuppressants (azathioprine, mycophenolate, cyclophosphamide) or biologics (belimumab, rituximab).
Self-care is also very important:
- Understand and identifying what triggers your flares and avoid them
- Keep your regular scheduled appointments, and maintain your treatment plan until your healthcare provider and you decide to make changes.
- Maintain a healthy lifestyle, including a good diet, exercise and try to reduce your stress.
- Report any new or worsening chest or abdominal pain promptly!
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Long-Term Outlook
Most patients with lupus-related serositis do well with treatment. The symptoms usually resolve without lasting complications. However, untreated or severe inflammation may result in:
- Pleural effusion, a significant fluid around lungs for long periods of time..
- Pericardial effusion or tamponade, dangerous fluid around the heart.
- Constrictive pericarditis, the rare scarring of the heart lining.
Close monitoring and regular communication with healthcare providers are crucial for preventing these complications.
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In Conclusion
Serositis is a significant yet often overlooked condition of lupus. Though it can cause pain and distress, most cases are treatable with appropriate medications and monitoring.
Knowing your symptoms, especially chest or abdominal pain that worsens with movement or position, can help with a timely diagnosis.
With awareness, prompt treatment, and consistent management, lupus-related serositis does not have to stand in the way of living a full and active life.
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References
Bertsias, G., Cervera, R., & Boumpas, D. T. (2012). Systemic lupus erythematosus: pathogenesis and clinical features. In EULAR textbook on rheumatic diseases (pp. 476–505). BMJ Publishing Group.
Cervera, R., Khamashta, M. A., Font, J., Sebastiani, G. D., Gil, A., Lavilla, P., … & Hughes, G. R. (2003). Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients. Medicine, 82(5), 299–308.
Choi B.Y., Yoon M.J., Shin K., Lee Y.J., & Song Y.W. (2014). Characteristics of pleural effusions in systemic lupus erythematosus: differential diagnosis of lupus pleuritis. Lupus, 24(3):321-326. doi:10.1177/0961203314555171
Doria, A., Zen, M., Canova, M., Bettio, S., Bassi, N., Nalotto, L., & Ghirardello, A. (2018). SLE diagnosis and treatment: When early is early. Autoimmunity Reviews, 17(6), 618–622.
Petri, M., Orbai, A. M., Alarcón, G. S., Gordon, C., Merrill, J. T., Fortin, P. R., & Costenbader, K. H. (2012). Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis & Rheumatism, 64(8), 2677–2686.
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Author: Greg Dardis, MS
Professor Dardis was 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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