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Systemic Lupus Erythematosus (SLE)

Lupus afflicts approximately 5 million people worldwide. Learn more about this chronic illness and how it impacts those who receive the diagnosis.

Introduction

Systemic lupus erythematosus (SLE) is a chronic connective tissue disorder than can affect various structures, organs and systems of the body.  SLE is one of five types of lupus which include:

  • Cutaneous lupus: Lupus that affects the skin and includes discoid lupus erythematosus (DLE), acute cutaneous lupus (ACLE), subacute cutaneous lupus erythematosus (SCLE) and tumid lupus.
  • Drug-induced: Certain medications can cause lupus-like symptoms (or side effects) though the individual does not have lupus itself. Drugs that may cause these symptoms include some blood pressure medications, minocycline, some heart medications and anti-TNF, which is used to treat rheumatoid arthritis.
  • Neonatal lupus erythematosus: Newborns may develop neonatal lupus even if they are born to mothers who do not have lupus. This form of lupus presents on the skin and usually subsides without treatment.
  • Childhood lupus. Children can develop lupus and in typically the same manner as adults. Kidney disease is more frequent in children with lupus, however, and often needs more aggressive monitoring and treatment.

SLE is also considered an autoimmune disorder because it causes the body to create antibodies against its own tissues and organs as though they are foreign invaders. This constant battle can cause inflammation throughout the body, as well as damage specific systems or structures – sometimes unpredictably.

Some quick facts about lupus include:

  • Approximately five million people globally have lupus, and most, approximately 90% of are women.
  • Lupus typically occurs between the ages of 15 and 45 and is most often diagnosed during a woman’s childbearing years.
  • Men do get lupus though they often develop it later than women and it often takes longer to diagnose.
  • Certain ethnicities have an increased risk of developing lupus including Hispanics, Asians and African Americans.
  • There is no known cure for lupus.

Though there is no cure, advancements in technology, medicine and improvement in managing SLE over the last 50-plus years have helped to increase the survival rate from approximately 60% within three years of diagnosis to a survival rate of over 90% within five to ten years of diagnosis. An individual’s symptoms can wax and wane over time as they experience lupus flares. Some individuals, however, achieve low-disease activity or even go into remission.

Throughout this article we will use the terms “SLE” and “lupus” interchangeably.

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How can SLE affect the body systemically?

SLE can involve and negatively impact the following systems:

  • Brain and nervous system: Cognitive dysfunction, headaches and migraines, neuropathy, fibromyalgia and central nervous system inflammation are just some of the common ways the brain and nervous system are implicated in SLE.
  • Opthalmologic (eyes): Dry eyes and inflammation of the white part of the eye (episcleritis and scleritis), the middle layer of the eye (uvea) and the retina (retinitis) are common manifestations of eye involvement. An individual may have or develop Sjögren’s syndrome as an overlap disease.
  • Dermatologic (skin): Cutaneous lupus, rashes and lesions caused by the onset of photosensitivity are some ways that SLE can affect the skin.
  • Hematologic (blood): Anemia, low platelet counts, low white blood cell counts and blood clots resulting from deep venous thrombosis or pulmonary embolus are ways the blood may be implicated.
  • Cardiovascular system: Inflammation of the heart and cardiovascular system including myocarditis, endocarditis, pericarditis, hypertension and atherosclerosis can result from SLE.
  • Respiratory system: A respiratory system compromised by lupus can result in pulmonary infection, antiphospholipid syndrome, pleuritis and pulmonary hypertension.
  • Gastrointestinal system: If an individual has lupus and gastrointestinal involvement, they may experience colitis, Crohn’s, pancreatitis or ulcers.
  • Musculoskeletal system: Conditions such as arthritis, myopathy and arthralgia are common in SLE.
  • Bones: Individuals with lupus may be more prone to experiencing bone loss, fracture and osteoporosis.
  • Oral disease: Sjögren’s syndrome and the development of mouth ulcers are two of the main ways the mouth may be compromised in SLE.
  • Reproductive system: Women who have SLE may find it difficult to get pregnant due to menstrual dysfunction and infertility. Men with SLE may have autoimmune orchitis which can result in infertility due to the presence of anti-sperm antibodies.
  • Renal system (kidneys): Lupus nephritis – inflammation of the kidneys – is one of the main ways SLE can target the renal system, especially in children.

It is also important to note that vasculitis – inflammation of the lining of the blood vessels – can occur in virtually any place in the body including the systems and structures above. Though it is relatively rare in the general population, vasculitis can reportedly affect up to 35% of individuals with SLE.

What causes SLE?

Though researchers are uncertain as to what specifically causes lupus, they have determined that several factors may trigger SLE, including:

  • Genetics: Individuals with family members who have an autoimmune disease may be at greater risk for developing one themselves. George C. Tsokos and his peers, for example, found that if one of a pair of identical twins has lupus, the other twin is at significantly greater risk of developing it as well. Epigenetics – the study of changes in gene expression due to environmental factors – can also play a role in SLE.
  • Hormones: Estrogen may play a significant role in the development of lupus in women. The female immune system is characterized as more “responsive and robust” than the male immune system, and this may give women the tendency to have it become overly active – possibly leading to the development of autoimmune disease. The onset of puberty increases the onset of lupus and several studies show that estrogen can enhance the severity and frequency of lupus flares.
  • Antibodies: Researchers of a 2015 review published in the journal Autoimmunity Reviews discovered that 180 different antibodies have been found in individuals with SLE. This is the largest number of antibodies found in any autoimmune disease. These researchers also found that approximately 50% of individuals with lupus have antiphospholipid antibodies and antibodies against ß2-glycoprotein 1.
  • Digestive health and gut microbiome: Individuals with SLE have a much less diverse microbiome in the gut than individuals without SLE. Those with lupus are found to have a higher count of the gut bacteria Ruminococcus gnavus, especially those who have lupus nephritis as well.
  • Cellular Factors: Elevated levels of cytokines and B cells are often present in individuals with lupus. Treg cells may also play a role in the development of lupus as they can exacerbate the autoimmune response, which stimulates B cell production.
  • Epstein Barr virus (EBV): EBV may trigger SLE in some individuals by changing how B cells function. A study by Drs. John Harley, Leah Kottyan and Matthew Weirauch found that most children who develop SLE have been infected with EBV.
  • Medications: Drug-induced lupus occurs from the side effects of certain medications. While the individual may not actually have lupus, they have the symptoms. These symptoms typically go away after the medication is stopped.

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What are the symptoms of SLE?

Symptoms of SLE can vary from person-to-person – no two people will experience lupus in the exact same way. Symptoms also may vary depending on which part of the body or which system is most affected. In general, however, symptoms include:

  • Fatigue: Fatigue is different than feeling sleepy or tired. It can affect the whole body resulting in profound exhaustion that can be emotionally and physically debilitating.
  • Rash: Though a rash may occur anywhere on the body, the most prevalent with lupus is malar rash or “butterfly” rash that develops across the bridge of the nose and cheeks.
  • Lesions: If an individual has lupus, they may develop skin lesions as a result from sensitivity to UV exposure and photosensitivity to the sun.
  • Raynaud’s phenomenon: Raynaud’s is caused by the narrowing of the small arteries that supply blood to the skin. Fingers, toes or even the nose can grow exceedingly cold even when the environment is not. Raynaud’s can also change the color of skin from blue to red or even white.
  • Breathing problems and/or chest pain: Seemingly inexplicable shortness of breath or chest pain can occur in individual’s with lupus, which can be due to conditions such as pleuritis or acute lupus pneumonitis. Chest pain can also be caused by inflammation of the cartilage, chest muscles or ligaments that connect the ribs to the breastbone.
  • Dry eyes: Sergio Schwartzman notes that the most common eye symptom of lupus is dry eye and is present when there is inflammation of the lacrimal glands, which produce tears. Individuals with dry eye report experiencing feeling like they have sand in their eyes.
  • Headache and/or cognitive impairment: According to the Johns Hopkins Lupus Center, approximately 20% of individuals with SLE will experience migraine-like headaches that may require corticosteroids for treatment. Brain fog is also common and includes difficulties in remembering, focusing and planning.
  • Fever: Homa Timlin and others found that up to 86% of individuals with SLE experience fevers. Often, individuals experiencing increased disease activity or who have a low white blood cell count will run a fever as well.
  • Muscle and joint pain and weakness: Inflammation can cause achy and stiff muscles and joints. Individuals commonly report feeling this tenderness and swelling in their thighs, arms, shoulders and neck. At times, those with lupus can feel too weak to perform simple daily tasks.
  • Kidney problems: Lupus often presents as lupus nephritis or inflammation of the kidneys. Symptoms include swollen ankles, changes in kidney function, high blood pressure or inexplicable weight gain. Lupus nephritis can be a life-threatening condition.

Diagnosing SLE

A rheumatologist will typically diagnose lupus.

There is no one test for lupus. In 2019, the European League Against Rheumatism (EULAR) in partnership with the American College of Rheumatology (ACR) developed criteria for healthcare practitioners to assess an individual’s risk for having lupus. These criteria, in conjunction with multiple lab and imaging tests, can be used to diagnose lupus.

The EULAR/ACR criteria is as follows:

“The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified” with the diagnosis of SLE.

Lab and imaging tests may include:

  • Complete medical history: A healthcare practitioner will assess an individual’s prior and current health to check for symptoms that may indicate the presence of autoimmune disease.
  • Physical exam: Because of the vast diversity of symptoms in many parts of the body, an individual will be examined from head to toe to look for as many symptoms as possible.
  • Family history of autoimmunity: Family health history and the incidence of autoimmune disease can often be an indicator of active autoimmune issues in an individual.
  • Complete blood counts: These blood tests measure red blood cell, white blood cell and platelet counts. They also measure the amount of protein or hemoglobin found in red blood cells. It is common in lupus for blood counts to indicate anemia.
  • Erythrocyte sedimentation rate: Sed rate tests determine how fast red blood cells settle at the bottom of a test tube over an hour. The faster they settle, the higher the indication of some kind of systemic disease.
  • Kidney and liver assessment: A healthcare practitioner may take blood tests to assess liver and kidney function since lupus can affect both.
  • Urinalysis: Protein and/or blood in urine may indicate that lupus is present and has affected the kidneys.
  • Antinuclear Antibody (ANA) test: ANA is an antibody that indicates an overly stimulated immune system. The presence of ANA is not always an indicator of lupus, however, and may necessitate the need for further antibody testing.
  • C-reactive protein test: This protein often shows up when there is inflammation in the body.
  • Complement test: A complement is a group of proteins found in the blood that destroy foreign substances. When levels are low, it may be because of lupus.
  • Chest X-ray: Shadows in the lungs may indicate inflammation or the presence of fluid which often occur with lupus.
  • Echocardiogram: An echocardiogram tests checks heart beat and may indicate valve issues or problems with other parts of the heart.
  • Kidney biopsy: Since lupus can initially present in the kidneys, a healthcare practitioner may order a kidney biopsy to check for abnormalities in kidney function.
  • Skin biopsy: Much like a kidney biopsy, a healthcare practitioner will look at a skin sample to see if it indicates the presence of lupus.

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Managing and Treating SLE

A healthcare practitioner will work an individual who has lupus to develop a treatment plan that addresses the myriad of symptoms the individual may be experiencing. The plan will be individualized to the individual’s current needs and adjusted as symptoms change. There is no “one size fits all” treatment plan.

Commonly prescribed lupus medications include:

  • Immunosuppressives: These drugs suppress an overactive immune system and work to control inflammation. Examples of immunosuppressives include cyclophosphamide (Cytoxan), methotrexate (Rheumatrex) and azathioprine (Imuran).
  • Corticosteroids: Also known as glucocorticoids or steroids, this class of medicine work like cortisol and help to regulate the immune system and decrease inflammation. Common drugs include prednisone, prednisolone and methylprednisolone.
  • Antimalarials: Antimalarials are used in conjunction with steroids and other medications and are used to temper the doses of those other medications. They are also often given to treat rashes, mouth ulcers and joint pain. Hydroxychloroquine (Plaquenil) and chloroquine (Aralen) are types of antimalarials.
  • Monoclonal antibodies (mAbs): Belimumab (Benlysta) is a biologic and is the only prescribed medication in this drug class. It works to stop the interference of B lymphocytes with BLyS which is a protein needed for B cell activity.
  • Non-steroidal anti-inflammatories: These medications are available over-the-counter and by prescription. They work to reduce inflammation and joint stiffness and pain. Ibuprofen, naproxen and celecoxib are examples of these drugs.
  • Acetaminophen: Also known by the brand name Tylenol, acetaminophen is commonly prescribed to reduce pain.
  • Aspirin: Aspirin may be prescribed to treat pain, inflammation and thin blood.

Though somewhat controversial and lacking substantial research, medical cannabis may prove beneficial for treating some of the symptoms of lupus and overlap diseases such as rheumatoid arthritis or other medical conditions such as fibromyalgia. Medical cannabis may provide relief for anxiety, stress, pain, inflammation, nausea and poor appetite. It is strongly suggested, however, that an individual thoroughly discuss the pros and cons of this option with their healthcare practitioner before embarking on this form of therapy.

Individuals often seek relief though the use of complementary therapies and find them beneficial. These therapies include:

In order to help cope with the emotional effects of having a chronic illness and to foster greater well-being, a healthcare practitioner may suggest seeing a therapist who can help an individual manage their feelings and mental health through a modality such as cognitive behavioral therapy. Joining a lupus support group is also a great way to talk with others who are going through similar experiences and share the ups and downs of living with chronic illness. A holistic approach to treating and managing lupus takes the whole being into consideration and not just the physical manifestations of the illness.

Practicing healthy habits and a healthy lifestyle can also help overall disease management. These habits include:

  • Exercise: Getting enough exercise can help manage pain and stiffness as well as promote healthy sleep. A healthcare practitioner can help determine an appropriate fitness routine.
  • Maintaining a healthy weight: Maintaining a healthy weight can not only help manage lupus symptoms, but can also stave off other conditions such as diabetes or cardiovascular disease.
  • Eating a healthy diet: Eating a well-balanced diet and using supplements as recommended by a healthcare practitioner is imperative for managing chronic illness.
  • Limiting alcohol and caffeine intake: Caffeine can overstimulate emotions such as stress and anxiety and can also exacerbate conditions such as Raynaud’s phenomenon or irregular heartbeat. Alcohol can interact with drugs, negatively impact sleep, increase heart rate and cause dehydration.
  • Getting plenty of rest: Knowing when to slow down, stop or say no can ensure an individual gets adequate rest. Creating a healthy sleep environment and carving out time during the day to relax and rejuvenate can give an individual a sense of control over their health.

In Conclusion

While having a chronic health condition such as SLE can be overwhelming and undoubtedly impact daily life, an inclusive approach to well-being that includes medical intervention, emotional support and self-care can help an individual achieve the most optimal health possible. As more and more lupus awareness is cultivated and funding is secured, research efforts can expand and identify more targeted methods for diagnosing, treating or possibly even preventing lupus. Individuals with lupus are therefore encouraged to advocate for themselves and others with lupus. Those who do not have lupus – but know someone who does – are likewise encouraged to become allies for their friends and loved ones.

 

References

American Academy of Ophthalmology. (2017, May 27). I have lupus. Can this affect my vision? Most days my vision is blurred and I have to squint to read, even though I wear glasses. Also, sometimes when I drive or watch TV, I see double and need to move my head to focus. [Online forum post]. Ask an Ophthalmologist.https://www.aao.org/eye-health/ask-ophthalmologist-q/can-lupus-affect-my-vision

Arigner, M., Costenbader, K., Daikh, D., Brinks, R., Mosca, M., Ramsey-Goldman, R., Smolen, J., Wofsy, D., Boumpas, D., Kamen, D., Jayne, D., Cervera, R., Costedoat-Chalumeau, N., Diamond, B., Gladman, D., Hahn, B., Hiepe, F., Jacobsen, S., … Johnson, S. (2019). 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis & Rheumatology, (71)9, 1400-1413. doi: 10.1002/art.4093. Retrieved February 24, 2020 from https://www.rheumatology.org/Portals/0/Files/Classification-Criteria-Systemic-Lupus-Erythematosus.pdf

Azzouz, D., Omarebekova, A., Heguy, A., Schwudke, D., Gisch, N., Rovin, B., Caricchio, R., Buyon, J., Alekseyenko, A., & Silverman, G. (2018). Lupus nephritis is linked to disease-activity associated expansions and immunity to a gut commensal. Annals of the Rheumatic Diseases, (2019)78, 947-956. doi: 10.1136/annrheumdis-2018-214856. Retrieved February 24, 2020 from  https://ard.bmj.com/content/annrheumdis/78/7/947.full.pdf

Complications of lupus. (n.d.). Michigan Medicine of University of Michigan. Retrieved February 24, 2020 from https://www.uofmhealth.org/health-library/hw118452

Green, J. (n.d.). The link between lupus and teeth. Colgate. Retrieved February 24, 2020 from https://www.colgate.com/en-us/oral-health/conditions/immune-disorders/the-link-between-lupus-and-teeth-0515

Lupus primer. (2020). Johns Hopkins Lupus Center. Retrieved February 24, 2020 from https://www.hopkinslupus.org/lupus-info/

Rees, F., Doherty, M., Grainge, M., Lanyon, P., & Zhang, W. (2017). The worldwide incidence and prevalence of systemic lupus erythematosus: A systematic review of epidemiological studies. Rheumatology, (56)11, 1945-1961. doi: 10.1093/rheumatology/kex260. Retrieved February 24, 2020 from https://academic.oup.com/rheumatology/article/56/11/1945/4079913

Systemic lupus erythematosus. (2018). The Centers for Disease Control and Prevention. Retrieved February 24, 2020 from https://www.cdc.gov/lupus/facts/detailed.html

Systemic lupus erythematosus (SLE). (2014). The Oncofertility Consortium of Northwestern University. Retrieved February 24, 2020 from http://oncofertility.northwestern.edu/resources/systemic-lupus-erythematosus-sle

Systemic lupus erythematosus (SLE or lupus). (2020). University of Rochester Medical Center. Retrieved February 24, 2020 from (https://www.urmc.rochester.edu/encyclopedia/content.aspx?ContentTypeID=85&ContentID=P00058

Tomlin, H., Syed, A., Haque, U., Adler, B., Law, B., Machireddy, K., & Manno, R. (2018). Fevers in adult lupus patients. Cureus (10)1, Article e2098. doi: 10.7759/cureus.2098. Retrieved February 24, 2020 from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5866120/pdf/cureus-0010-00000002098.pdf

Tsokos, G., Lo, M., Costa Reis, P., & Sullivan, K. (2016). New insights into the immunopathogenesis of systemic lupus erythematosus. Nature, (2016)12, 716-730. doi: 10.1038/nrrheum.2016.186. Retrieved February 24, 2020 from https://autoimmunity-network.com/articles/sheba%205299.pdf

Villalba, M., & the staff of the National Institute of Arthritis and Musculoskeletal and Skin Diseases. (2019, March 25). Lupus and women. Office on Women’s Health. https://www.womenshealth.gov/lupus/lupus-and-women

Yaniv, G., Twig, G., Ben-Ami Shor, D., Furer, A., Sherer, Y., Mozes, O.,  Komisar, O., Slonimsky, E., Klang, E., Lotan, E., Welt, M., Marai, I., Shina, A., Amital, H., & Shoenfeld, Y. (2015). A volcanic explosion of autoantibodies in systemic lupus erythematosus: A diversity of 180 different antibodies found in SLE patients. Autoimmunity Reviews, (14)1, 75-79. doi: 101016/j.autrev.2014.10.003.

 

Author: Liz Heintz

Liz Heintz is a technical and creative writer who received her BA in Communications, Advocacy, and Relational Communications from Marylhurst University in Lake Oswego, Oregon. She most recently worked for several years in the healthcare industry. A native of San Francisco, California, Liz now calls the beautiful Pacific Northwest home.

All images unless otherwise noted are property of and were created by Kaleidoscope Fighting Lupus. To use one of these images, please contact us at [email protected] for written permission; image credit and link-back must be given to Kaleidoscope Fighting Lupus.

All resources provided by us are for informational purposes only and should be used as a guide or for supplemental information, not to replace the advice of a medical professional. The personal views expressed here do not necessarily encompass the views of the organization, but the information has been vetted as a relevant resource. We encourage you to be your strongest advocate and always contact your healthcare practitioner with any specific questions or concerns.

 

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