Systemic Lupus Erythematosus

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Vol. 1 No. 1 (2024)

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Submitted
May 23, 2024
Published
July 31, 2024

Systemic Lupus Erythematosus - Causes, Symptoms and Treatment Options

https://doi.org/10.69512/ajphi.v1i01.39
Jwala KC, MD
Department of Pediatrics, Western Michigan University Homer Stryker MD School of Medicine, Kalamazoo, MI, USA
Sujata Maharjan, MD
Chitwan Medical College, Chitwan, Nepal

Corresponding Author(s) : Jwala KC, MD

kc.jwala2019@gmail.com

The American Journal of Patient Health Info, Vol. 1 No. 1 (2024)

Abstract


  1. Systemic lupus erythematosus is a chronic autoimmune disease where the body's immune system mistakenly attacks its own tissues and organs. 

  2. Common symptoms include fatigue, joint pain and swelling, skin rashes (especially the butterfly rash on the face), fever, chest pain, and sensitivity to sunlight.

  3. Diagnosis typically involves a combination of medical history, physical examination, laboratory tests (such as blood tests for antibodies like ANA), and imaging studies.

  4. Treatment for systemic lupus erythematosus involves a combination of medications to control symptoms and minimize organ damage, alongside lifestyle changes like sun protection and exercise.

  5. SLE can lead to various complications, including kidney damage (lupus nephritis), cardiovascular problems, neurological disorders, osteoporosis, and increased susceptibility to infections.

Keywords

Antinuclear Antibody Corticosteroid Lupus SLE Pregnancy
KC, MD, J., & Maharjan, MD, S. (2024). Systemic Lupus Erythematosus - Causes, Symptoms and Treatment Options. The American Journal of Patient Health Info, 1(1). https://doi.org/10.69512/ajphi.v1i01.39
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References
  1. Izmirly PM, Parton H, Wang L, McCune WJ, Lim SS, Drenkard C, et al. Prevalence of Systemic Lupus Erythematosus in the United States: Estimates From a Meta‐Analysis of the Centers for Disease Control and Prevention National Lupus Registries. Arthritis & Rheumatology 2021;73(6):991–6.
  2. Megan R.W. Barber, Titilola Falasinnu, Ramsey-Goldman R, Clarke AE. The global epidemiology of SLE: narrowing the knowledge gaps. Rheumatology. 2023 Mar 29;62(Supplement_1):i4–9. Available from: https://academic.oup.com/rheumatology/article/62/Supplement_1/i4/7087226
  3. Solhjoo M, Bansal P, Goyal A, Chauhan K. Drug-Induced Lupus Erythematosus. PubMed. Treasure Island (FL): StatPearls Publishing; 2020. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441889/
  4. Egner W. The use of laboratory tests in the diagnosis of SLE. Journal of Clinical Pathology. 2000 Jun 1;53(6):424–32.
  5. Porta S, Danza A, Arias Saavedra M, Carlomagno A, Goizueta MC, Vivero F, et al. Glucocorticoids in Systemic Lupus Erythematosus. Ten Questions and Some Issues. J Clin Med 2020;9(9):2709.
  6. Katarzyna Pawlak-Buś, Schmidt W, Dudziec Ewa, Leszczyński Piotr. Current treatment of systemic lupus erythematosus: a clinician’s perspective. Rheumatology International. 2023 Aug 1;43(8):1395–407.
  7. Corbin D, Christian L, Rapp CM, Liu L, Rohan CA, Travers JB. New concepts on abnormal UV reactions in systemic lupus erythematosus and a screening tool for assessment of photosensitivity. Skin Research and Technology 2023;29(3).
  8. Nikolopoulos D, Parodis I. Janus kinase inhibitors in systemic lupus erythematosus: implications for tyrosine kinase 2 inhibition. Front Med (Lausanne) 2023;10.
  9. Parodis I, Gatto M, Sjöwall C. B cells in systemic lupus erythematosus: Targets of new therapies and surveillance tools. Front Med (Lausanne) 2022;9. 10. flare up prevention
  10. Frade S, O’Neill S, Greene D, Cameron M. Exercise as adjunctive therapy for systemic lupus erythematosus. Cochrane Database of Systematic Reviews 2021;2021(10).
  11. NIAMS. Systemic Lupus Erythematosus (Lupus). National Institute of Arthritis and Musculoskeletal and Skin Diseases. 2017. Available from: https://www.niams.nih.gov/health-topics/lupus/diagnosis-treatment-and-steps-to-take
  12. Iozza I, Cianci S, Di Natale A, Garofalo G, Giacobbe AM, Giorgio E, et al. Update on systemic lupus erythematosus pregnancy. Journal of prenatal medicine . 2010;4(4):67–73. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186/
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Jwala KCa, Sujata Maharjanb

a Department of Pediatrics, Western Michigan University Homer Stryker MD School of Medicine, Kalamazoo, MI, USA

b Chitwan Medical College, Chitwan, Nepal

Background

Systemic lupus erythematosus (SLE), also commonly known as lupus, is a chronic autoimmune disease. This means that your immune system cannot distinguish between healthy tissue and foreign invaders and attacks healthy cells and tissues by mistake. The inflammation may affect the skin, joints, heart, lungs, kidneys, circulating blood cells, brain, and other organs and, in some cases, permanent tissue damage.

In the United States, the National Lupus Registry showed a prevalence of SLE to be approximately 72.8 per 100,000 person-years, with a higher prevalence among females (128.7 per 100,000) compared to males (14.6 per 100,000). The prevalence is notably higher among Black females (230.9 per 100,000) and American Indian/Alaska Native females (270.6 per 100,000). 1

Causes and Risk Factors of Systemic Lupus Erythematosus

SLE has no singular cause, but it's influenced by various factors:

Figure 1.

  • Ethnicity and Gender: People of all ages, including children, are susceptible to SLE. However, the most significant risk of getting SLE is among women of reproductive age (15 to 44). Women are affected more than males (9:1), regardless of age. Compared to white women, SLE affects African American women two to three times more frequently. Additionally, it affects Hispanic, Asian, and Native American women more frequently. African American or Hispanic women are more likely to have severe lupus. 2
  • Immune System Dysfunction: Abnormalities in the immune system, including overactivity or dysregulation, contribute to the development of SLE.
  • Hormonal Factors: Hormonal changes, particularly in women, such as during puberty, pregnancy, or menopause, can affect SLE activity.
  • Environmental Triggers: Exposure to certain environmental factors, like infections and sunlight, may initiate or exacerbate SLE.
  • Medications: Certain medications like Isoniazid, Hydralazine, Procainamide, Minocycline, Quinidine, and (TNF)Alpha Inhibitors such as Infliximab, Adalimumab, Etanercept exacerbates SLE. 3

Symptoms of Systemic Lupus Erythematosus

Almost every organ in your body is at risk for SLE, Lupus symptoms typically vary, so you don't always experience them. Symptoms may come and go. When you are having worsening symptoms, it is called a flare. Flares can range from mild to severe and are unpredictable. Symptoms of lupus can include:

Figure 2.

  • Joint and Muscle Discomfort: You might feel stiffness and soreness together with or without swelling. Most lupus sufferers are affected by this. The neck, legs, shoulders, and upper arms are typical locations for muscular soreness and swelling.
  • Fever: Many people with lupus experience a temperature above 100 F. Fever is frequently brought on by illness or inflammation. Fever can be managed and prevented with lupus medication.
  • Rash: Any area of your body exposed to the sun, such as your face, arms, and hands, is susceptible to developing rashes. A crimson, butterfly-shaped rash that covers the nose and cheeks is one typical lupus symptom.
  • Sensitive to the Sun or Light: Most lupus sufferers experience photosensitivity or sensitivity to light. Some people with lupus may get rashes, a fever, extreme exhaustion, or joint discomfort after sunlight exposure.
  • Hair Loss: Bald or patchy areas are typical. Some medications or infections might also result in hair loss.
  • Kidney Problems: Lupus nephritis affects the kidneys and affects 50% of people with lupus. Symptoms include weight gain, swollen ankles, elevated blood pressure, and impaired renal function.
  • Mouth Sores: These sores, also known as ulcers, typically develop on the mouth's roof but can also develop on the gums, inside the cheeks, and on the lips. You might have discomfort or dry mouth, or they could be painless.
  • Anemia: Anemia, a disorder that occurs when your body lacks red blood cells to transport oxygen throughout your body, might cause fatigue.
  • Blood Clots: Your risk of blood clotting may be more significant. Blood clots increase the risk of a heart attack, a stroke, or recurrent miscarriages.

Diagnosis of Systemic Lupus Erythematosus

As a result of its many irregular symptoms and ability to resemble those of other diseases or disorders, lupus can be challenging to diagnose. A single blood test cannot currently diagnose lupus. Before lupus is identified, your doctor may need to rule out other potential reasons for the symptoms.

Figure 3.

  • Medical History: The doctor will inquire about symptoms, family history, and any previous medical conditions or treatments.
  • Physical Examination: A thorough examination may reveal characteristic symptoms like skin rashes, joint swelling, or other signs of organ involvement.
  • Laboratory Tests: Blood tests can detect specific antibodies associated with SLE, such as antinuclear antibodies (ANA), anti-double-stranded DNA (anti-dsDNA) antibodies, and anti-Smith antibodies. Additionally, tests for inflammation markers and organ function help assess disease activity and potential complications. 4
  • Imaging Studies: Imaging tests like X-rays, ultrasounds, or Computed Tomography scans may be used to evaluate organ damage or abnormalities.
  • Biopsy: In some cases, a tissue biopsy of affected organs, such as the skin, kidney, or liver, may be necessary to confirm the diagnosis and assess the extent of organ involvement.

Treatment of Systemic Lupus Erythematosus

Treatment for  SLE aims to manage symptoms, prevent flare-ups, and minimize organ damage. It typically involves a combination of medications and lifestyle modifications:

Figure 4.

1. Medications

  • Nonsteroidal Anti-Inflammatory Drugs (NSAIDs): Help relieve pain and inflammation.
  • Corticosteroids: Reduce inflammation and suppress the immune system during flare-ups.5
  • Immunosuppressants: Methotrexate, Azathioprine, or mycophenolate mofetil help control the overactive immune response. 6
  • Antimalarial Drugs: Drugs like hydroxychloroquine, help manage skin rashes, joint pain, and fatigue while reducing the risk of flare-ups.

2. Lifestyle Modifications

  • Sun Protection: Avoid direct sunlight and use sunscreen to prevent skin rashes and photosensitivity. 7
  • Healthy Diet: Consuming a balanced diet of fruits, vegetables, whole grains, and lean proteins may help manage symptoms and reduce inflammation.
  • Regular Exercise: Promotes cardiovascular health, maintains joint flexibility, and improves overall well-being.
  • Stress Management: Stress reduction techniques such as mindfulness, meditation, or counseling can help manage psychological symptoms and potentially reduce disease flares.

3. Monitoring and Follow-up

Regular monitoring by healthcare providers is essential to assess disease activity, monitor medication side effects, and detect potential complications. Based on disease activity, symptoms, and individual response to medications, adjustments to treatment may be necessary.

What’s New in the Treatment of Systemic Lupus Erythematosus?

Newer treatments are being studied for the treatment of SLE, with some showing beneficial effects.

  • Janus Kinase Inhibitors: They target the JAK-STAT signaling pathway, which is implicated in the pathogenesis of SLE.  These drugs include Tofacitinib  (JAK1/3 inhibitor), Baricitinib (JAK1/2 inhibitor), and Deucravacitinib (selective TYK2 inhibitor). They have shown benefits in reducing cholesterol levels, improving vascular function, and improving lupus rashes. 8
  • B-Cell Depletion Therapy: B-cell depletion is a well-established approach in SLE due to the central role of B cells in autoantibody production and immune regulation. Rituximab, an anti-CD20 monoclonal antibody, has been used off-label with mixed results in clinical trials. Belimumab, targeting B-lymphocyte stimulator (BLyS), is approved for SLE and has shown efficacy in non-renal lupus and lupus nephritis. Other agents like atacicept and epratuzumab have shown potential but require further validation. 9

Prevention of flare-ups of Systemic Lupus Erythematosus

Firstly, identify the symptoms of a flare-up. Spotting the warning signs of a flare can help you prevent the flare or make the symptoms less severe. You and your doctor can take steps to lessen or avoid it.

Figure 5.

  • Protect yourself from the sun-it is important since it can occasionally induce a flare. Wear protective clothing like a hat or long sleeves when you go outside, and apply sunscreen when outside the house. 10
  • Exercise regularly, find ways to relax through deep breathing, reading, or meditation, and reduce tension. Finding ways to cope with the stress of having lupus is also essential. Keep in mind that stress might cause an attack. 11
  • Consume a nutritious, balanced diet of whole grains, fruits, and vegetables.
  • Learn to recognize the symptoms of a flare-up so that you and your doctor can take steps to lessen or prevent it. The warning signs of lupus flare may include increased tiredness, joint swelling, pain, rash, fever, abdominal pain, or a headache.

Complications of Systemic Lupus Erythematosus

SLE can lead to various complications affecting multiple organs and systems in the body:

Figure 6.

  • Kidney Damage (Lupus Nephritis): One of the most serious complications of SLE, lupus nephritis causes inflammation and damage to the kidneys, leading to impaired kidney function and potentially kidney failure.
  • Cardiovascular Complications: SLE increases the risk of cardiovascular diseases such as heart attacks, strokes, and inflammation of the heart or surrounding tissues (pericarditis).
  • Neurological Disorders: SLE can affect the nervous system, leading to conditions like seizures, headaches, cognitive dysfunction (brain fog), peripheral neuropathy, or psychiatric disorders like anxiety and depression.
  • Pulmonary Complications: SLE may cause lung inflammation (pleuritis), leading to chest pain and difficulty breathing. In severe cases, it can result in pulmonary hypertension or interstitial lung disease.
  • Joint Damage (Arthritis): Chronic joint inflammation can cause arthritis, leading to pain, stiffness, and reduced mobility. In some cases, joint damage can be irreversible. 12
  • Skin and Mucosal Manifestations: SLE commonly presents skin rashes, such as the characteristic butterfly rash on the face, discoid lupus lesions, photosensitivity, and mucosal ulcers in the mouth and nose.
  • Osteoporosis: Chronic inflammation, medications like corticosteroids, and decreased physical activity can increase the risk of osteoporosis and fractures in people with SLE.
  • Infections: SLE and its treatment, particularly immunosuppressive medications, can weaken the immune system, making individuals more susceptible to infections.
  • Reproductive Complications: SLE can increase the risk of pregnancy complications, including miscarriage, preterm birth, preeclampsia, and neonatal lupus in newborns.
  • Increased Cancer Risk: People with SLE have a slightly higher risk of certain cancers, particularly non-Hodgkin lymphoma.

Pregnancy and Contraception for Women with Lupus

Most women who have SLE can give birth to healthy babies If their lupus is under control. Consult your doctor if you consider getting pregnant to ensure you are as healthy as possible before giving birth. Close monitoring during pregnancy is imperative if you have low platelets, antiphospholipid antibodies, anti-SSA/Ro antibodies, high blood pressure, or lung, heart, or renal issues. 12

Certain lupus drugs are contraindicated during pregnancy. To ensure that all your lupus drugs are safe during pregnancy, discussing them with your doctors before becoming pregnant is crucial. Birth control pills do not increase the incidence of severe flares among lupus patients; however, estrogen-containing drugs are not advised for those with antiphospholipid antibodies. Before beginning oral contraceptives, discuss your antibody test results with your doctor.

Questions To Ask Your Doctor

  1. What treatment options for SLE are good for me, and how effectively are they managing the symptoms?
  2. What lifestyle changes can I make to improve their quality of life and manage symptoms?
  3. Are any new treatments or therapies being developed for SLE?
  4. What kind of follow-up care do I need?
  5. Are there any support groups or resources available for patients with SLE and their families?

References

  1. Izmirly PM, Parton H, Wang L, McCune WJ, Lim SS, Drenkard C, et al. Prevalence of Systemic Lupus Erythematosus in the United States: Estimates From a Meta‐Analysis of the Centers for Disease Control and Prevention National Lupus Registries. Arthritis & Rheumatology 2021;73(6):991–6. Available from: https://pubmed.ncbi.nlm.nih.gov/33474834/
  2. Megan R.W. Barber, Titilola Falasinnu, Ramsey-Goldman R, Clarke AE. The global epidemiology of SLE: narrowing the knowledge gaps. Rheumatology. 2023 Mar 29;62(Supplement_1):i4–9. Available from: https://academic.oup.com/rheumatology/article/62/Supplement_1/i4/7087226
  3. Solhjoo M, Bansal P, Goyal A, Chauhan K. Drug-Induced Lupus Erythematosus. PubMed. Treasure Island (FL): StatPearls Publishing; 2020. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441889/
  4. Egner W. The use of laboratory tests in the diagnosis of SLE. Journal of Clinical Pathology. 2000 Jun 1;53(6):424–32. Available from: https://pubmed.ncbi.nlm.nih.gov/10911799/
  5. Porta S, Danza A, Arias Saavedra M, Carlomagno A, Goizueta MC, Vivero F, et al. Glucocorticoids in Systemic Lupus Erythematosus. Ten Questions and Some Issues. J Clin Med 2020;9(9):2709. Available from: https://pubmed.ncbi.nlm.nih.gov/32839376/
  6. Katarzyna Pawlak-Buś, Schmidt W, Dudziec Ewa, Leszczyński Piotr. Current treatment of systemic lupus erythematosus: a clinician’s perspective. Rheumatology International. 2023 Aug 1;43(8):1395–407. Available from: https://pubmed.ncbi.nlm.nih.gov/37171669/
  7. Corbin D, Christian L, Rapp CM, Liu L, Rohan CA, Travers JB. New concepts on abnormal UV reactions in systemic lupus erythematosus and a screening tool for assessment of photosensitivity. Skin Research and Technology 2023;29(3). Available from: https://pubmed.ncbi.nlm.nih.gov/36973991/
  8. Nikolopoulos D, Parodis I. Janus kinase inhibitors in systemic lupus erythematosus: implications for tyrosine kinase 2 inhibition. Front Med (Lausanne) 2023;10. Available from: https://pubmed.ncbi.nlm.nih.gov/37457579/
  9. Parodis I, Gatto M, Sjöwall C. B cells in systemic lupus erythematosus: Targets of new therapies and surveillance tools. Front Med (Lausanne) 2022;9. 10. flare up prevention. Available from: https://pubmed.ncbi.nlm.nih.gov/36111105/
  10. Frade S, O’Neill S, Greene D, Cameron M. Exercise as adjunctive therapy for systemic lupus erythematosus. Cochrane Database of Systematic Reviews 2021;2021(10). Available from: https://pubmed.ncbi.nlm.nih.gov/37073886/
  11. NIAMS. Systemic Lupus Erythematosus (Lupus). National Institute of Arthritis and Musculoskeletal and Skin Diseases. 2017. Available from: https://www.niams.nih.gov/health-topics/lupus/diagnosis-treatment-and-steps-to-take
  12. Iozza I, Cianci S, Di Natale A, Garofalo G, Giacobbe AM, Giorgio E, et al. Update on systemic lupus erythematosus pregnancy. Journal of prenatal medicine . 2010;4(4):67–73. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3279186/

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