Systemic Lupus Erythematosus: Symptoms, Diagnosis & Treatment Guide

Introduction

Systemic lupus erythematosus (SLE), commonly referred to as lupus, is a chronic autoimmune disease that can affect various parts of the body, including the skin, joints, kidneys, brain, and other organs. First identified in the 19th century, our understanding of lupus has advanced significantly over time. In lupus, the immune system mistakenly targets healthy tissues, leading to inflammation and damage. The severity of the condition can vary widely, from mild to life-threatening, making early diagnosis and treatment crucial.

This article offers a comprehensive overview of lupus, discussing its risk factors, symptoms, diagnostic tests, treatment options, and lifestyle adjustments that can help manage the disease. Whether you have recently been diagnosed or are seeking more information, this guide aims to help you better understand lupus and how to live with it.

What is Systemic Lupus Erythematosus?

Systemic lupus erythematosus (SLE) is an autoimmune disease influenced by factors such as genetics and environmental triggers. It presents with a wide range of symptoms and can be managed through diagnostic tests, medications, procedures, and lifestyle changes.

Description of Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder in which the immune system, which normally defends the body against infections, mistakenly attacks its own tissues. This abnormal immune response can lead to widespread inflammation and damage in various organs, including the skin, joints, kidneys, heart, lungs, and brain. Lupus is known for its unpredictable nature, with periods of flares (when symptoms worsen) and remissions (when symptoms improve).

The progression of lupus varies significantly from person to person. Some individuals may experience mild symptoms, such as fatigue and joint pain, while others may face more severe complications, including kidney or heart issues. Lupus can also impact mental health, contributing to conditions like depression and anxiety.

According to the Lupus Foundation of America, approximately 1.5 million Americans live with lupus, with about 90% of those diagnosed being women. The disease most commonly affects individuals between the ages of 15 and 45, though it can occur at any age. Lupus is also more prevalent in certain ethnic groups, including African American, Hispanic, Asian, and Native American populations.

Risk Factors for Developing Systemic Lupus Erythematosus

Lifestyle Risk Factors

While lifestyle factors do not directly cause lupus, certain behaviors and environmental exposures can increase the risk of developing the disease or trigger flare-ups in those already diagnosed. For instance, exposure to ultraviolet (UV) light from the sun or artificial sources can provoke skin rashes and other lupus symptoms. Smoking has also been associated with an increased risk of developing lupus and can exacerbate symptoms in those already diagnosed. Additionally, stress is known to worsen lupus symptoms, as it can weaken the immune system and lead to flare-ups.

Medical Risk Factors

Certain medical conditions and treatments may heighten the likelihood of developing lupus. For example, individuals with other autoimmune diseases, such as rheumatoid arthritis or Sjögren’s syndrome, may have a higher risk of developing lupus. Some infections, like the Epstein-Barr virus (EBV), have also been linked to an increased risk of lupus. Additionally, certain medications, including specific antibiotics and anti-seizure drugs, can cause drug-induced lupus, a condition that mimics lupus but typically resolves once the medication is discontinued.

Genetic and Age-Related Risk Factors

Genetics play a significant role in the development of lupus. People with a family history of lupus or other autoimmune diseases are at a higher risk of developing the condition. While lupus can occur at any age, it is most commonly diagnosed in women of childbearing age (15-45 years). Women are about nine times more likely to develop lupus than men. Certain ethnic groups, such as African Americans, Hispanics, and Asians, are also at a higher risk, suggesting a genetic predisposition linked to ancestry.

Although these risk factors can increase the likelihood of developing lupus, having one or more does not guarantee that someone will develop the disease. Lupus is a complex condition with multiple contributing factors, and ongoing research continues to explore its causes and triggers.

Common Lupus Symptoms and Clinical Manifestations

Fatigue (80-100%)

Fatigue is one of the most common and debilitating symptoms of systemic lupus erythematosus (SLE), affecting up to 80-100% of patients. It can range from mild tiredness to extreme exhaustion, often unrelated to physical activity. SLE-related fatigue is believed to be caused by chronic inflammation, which affects multiple organs and tissues. This inflammation leads to an overproduction of cytokines, proteins that regulate immune responses, contributing to feelings of tiredness. Fatigue can worsen during lupus flares and may be exacerbated by anemia, sleep disturbances, or co-existing conditions like fibromyalgia.

Joint Pain (90%)

Joint pain, or arthralgia, occurs in about 90% of SLE patients. The pain typically affects smaller joints, such as those in the hands, wrists, and knees. Unlike rheumatoid arthritis, SLE-related joint pain is usually non-erosive, meaning it doesn’t cause permanent joint damage. Inflammation from lupus can cause the immune system to attack the synovium, the lining of the joints, leading to pain, stiffness, and swelling. Joint pain in lupus can vary in intensity and may come and go, often worsening during disease flares.

Skin Rash (70%)

Approximately 70% of people with lupus develop some form of skin rash, with the most characteristic being the “butterfly rash” across the cheeks and nose. This rash, also known as a malar rash, is typically red or purplish and may appear after sun exposure. Lupus can cause the immune system to attack skin cells, leading to inflammation and rashes. These rashes can be painful or itchy and may worsen with exposure to ultraviolet (UV) light, a phenomenon known as photosensitivity.

Fever (50-60%)

Fever is a common symptom in lupus, affecting 50-60% of patients, especially during disease flares. A low-grade fever (100°F to 102°F) is often a sign of inflammation or infection. In lupus, the immune system mistakenly attacks the body’s own tissues, leading to widespread inflammation that can cause fever. Persistent fever in lupus patients may also indicate an underlying infection, as lupus and its treatments can weaken the immune system, making infections more likely.

Hair Loss (45-55%)

Hair loss, or alopecia, affects 45-55% of people with lupus. It can occur due to inflammation in the scalp or as a side effect of lupus medications like corticosteroids. Lupus-related hair loss can be diffuse (thinning across the scalp) or localized, particularly in areas affected by skin rashes or lesions. In some cases, hair loss may be temporary and improve with treatment, but scarring from skin lesions can lead to permanent hair loss.

Photosensitivity (40-60%)

Photosensitivity, or an abnormal reaction to sunlight, affects 40-60% of lupus patients. Exposure to UV light can trigger or worsen lupus symptoms, including skin rashes, fatigue, and joint pain. The immune system in lupus patients may react to UV light by producing autoantibodies, which attack healthy skin cells. This can result in inflammation and damage to the skin, leading to rashes or lesions. Lupus patients are often advised to avoid prolonged sun exposure and use sunscreen to prevent flare-ups.

Mouth Ulcers (25-50%)

Mouth ulcers, also known as oral ulcers, occur in 25-50% of lupus patients. These ulcers are typically painless and can appear on the roof of the mouth, inside the cheeks, or on the gums. Lupus-related inflammation can affect the mucous membranes, leading to the development of these sores. While they are usually not painful, mouth ulcers can be a sign of a lupus flare and may be associated with other symptoms like fatigue or joint pain.

Swollen Lymph Nodes (30-40%)

Swollen lymph nodes, or lymphadenopathy, affect 30-40% of people with lupus. The lymph nodes are part of the immune system and can become enlarged when the body is fighting an infection or inflammation. In lupus, the immune system’s overactivity can cause the lymph nodes to swell as they respond to chronic inflammation. Swollen lymph nodes in lupus are usually painless and may come and go, often coinciding with disease flares.

Chest Pain (30-50%)

Chest pain, or pleuritic pain, is reported in 30-50% of lupus patients. This pain is often caused by inflammation of the lining around the lungs (pleuritis) or the heart (pericarditis). In lupus, the immune system can attack these linings, leading to inflammation and fluid buildup, which causes sharp, stabbing pain that worsens with deep breaths or coughing. Chest pain in lupus can be a sign of more serious complications, such as lupus-related heart or lung disease, and should be evaluated by a healthcare provider.

Kidney Problems (40-50%)

Kidney problems, or lupus nephritis, affect 40-50% of lupus patients. Lupus nephritis occurs when the immune system attacks the kidneys, causing inflammation and damage to the kidney’s filtering units (glomeruli). This can lead to symptoms like swelling in the legs, high blood pressure, and foamy urine due to protein leakage. If left untreated, lupus nephritis can progress to kidney failure, making early detection and treatment crucial. Regular monitoring of kidney function is important for lupus patients to prevent long-term complications.

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Health Conditions with Similar Symptoms to Systemic Lupus Erythematosus

Rheumatoid Arthritis

Rheumatoid arthritis (RA) is an autoimmune disease that primarily targets the joints, leading to inflammation, pain, and swelling, often in the hands, wrists, and knees. Over time, RA can cause joint deformity and loss of function. Unlike osteoarthritis, which results from wear and tear, RA occurs when the immune system mistakenly attacks the body’s tissues.

How to Know if You Might Have Rheumatoid Arthritis vs. Systemic Lupus Erythematosus

Both RA and systemic lupus erythematosus (SLE) can cause joint pain, stiffness, and swelling, making them difficult to distinguish. However, RA typically affects joints symmetrically, meaning both sides of the body are impacted similarly (e.g., both wrists or knees). Lupus, on the other hand, may cause less symmetrical joint pain. Additionally, lupus often presents with a broader range of symptoms, such as skin rashes (like the butterfly-shaped rash on the face), kidney problems, and sun sensitivity, which are not typical of RA.

Blood tests can help differentiate the two. In RA, tests for rheumatoid factor (RF) or anti-cyclic citrullinated peptide (anti-CCP) antibodies are often positive. In lupus, blood tests may show antinuclear antibodies (ANA), anti-dsDNA, or anti-Smith antibodies. X-rays or other imaging tests may reveal more joint damage in RA, especially if the disease has progressed.

Sjögren’s Syndrome

Sjögren’s syndrome is another autoimmune disorder that primarily affects moisture-producing glands, leading to dry eyes and dry mouth. It can also affect joints, skin, and organs. Like lupus, Sjögren’s syndrome is more common in women and can occur on its own or alongside other autoimmune diseases.

How to Know if You Might Have Sjögren’s Syndrome vs. Systemic Lupus Erythematosus

Both Sjögren’s syndrome and lupus can cause joint pain, fatigue, and skin rashes. However, Sjögren’s hallmark symptoms are dry eyes (gritty feeling or burning) and dry mouth (difficulty swallowing or speaking due to lack of saliva). While lupus can cause dryness, it is not as prominent or specific to moisture-producing glands as in Sjögren’s syndrome.

Blood tests can help differentiate the two. In Sjögren’s, anti-Ro/SSA and anti-La/SSB antibodies are often present. While these antibodies can also be found in some lupus patients, they are more common in Sjögren’s. A Schirmer’s test, which measures tear production, or a salivary gland biopsy can confirm Sjögren’s syndrome. In lupus, tests would more likely show elevated ANA, anti-dsDNA, or anti-Smith antibodies, along with systemic signs of organ involvement.

Dermatomyositis

Dermatomyositis is an inflammatory disease affecting muscles and skin. It causes muscle weakness, particularly in the shoulders, hips, and thighs, leading to difficulty climbing stairs, lifting objects, or swallowing. The condition also causes a distinctive skin rash, often appearing on the face, knuckles, and other sun-exposed areas.

How to Know if You Might Have Dermatomyositis vs. Systemic Lupus Erythematosus

Both dermatomyositis and lupus can cause skin rashes and muscle weakness, making them difficult to distinguish. However, the rash in dermatomyositis is more specific, often appearing as a purple or red discoloration on the eyelids (heliotrope rash) or raised, scaly patches on the knuckles (Gottron’s papules). Muscle weakness is more prominent in dermatomyositis, whereas lupus may cause muscle pain but not necessarily weakness.

Blood tests for muscle enzymes, such as creatine kinase (CK) and aldolase, are often elevated in dermatomyositis but not in lupus. A muscle biopsy or electromyography (EMG) can confirm muscle inflammation in dermatomyositis. In lupus, the focus would be on antibody tests like ANA, anti-dsDNA, or anti-Smith, and organ involvement such as kidney or lung issues.

Scleroderma

Scleroderma is a group of autoimmune diseases that cause hardening and tightening of the skin and connective tissues. It can affect only the skin (localized scleroderma) or involve internal organs such as the lungs, heart, and kidneys (systemic scleroderma). The disease occurs when the immune system causes an overproduction of collagen, leading to thickened skin and tissue damage.

How to Know if You Might Have Scleroderma vs. Systemic Lupus Erythematosus

Both scleroderma and lupus can cause joint pain, fatigue, and skin changes. However, the skin involvement in scleroderma is more specific, often leading to thickened, shiny skin, particularly on the hands and face. In lupus, skin rashes like the butterfly rash are more common, but the skin does not typically thicken. Scleroderma can also cause Raynaud’s phenomenon (fingers and toes turning white or blue in response to cold or stress), which can also occur in lupus but is more strongly associated with scleroderma.

Blood tests for specific antibodies, such as anti-centromere or anti-Scl-70 (anti-topoisomerase), can help diagnose scleroderma. In lupus, ANA is usually positive, but other antibodies like anti-dsDNA or anti-Smith are more specific. Imaging tests, such as a high-resolution CT scan, may show lung involvement in scleroderma, while lupus may show more kidney or heart issues.

Vasculitis

Vasculitis refers to a group of diseases that cause inflammation of the blood vessels. This inflammation can restrict blood flow, leading to damage in organs and tissues. Vasculitis can affect any part of the body, including the skin, joints, kidneys, lungs, and nervous system. Symptoms vary depending on which organs are involved.

How to Know if You Might Have Vasculitis vs. Systemic Lupus Erythematosus

Both vasculitis and lupus can cause joint pain, fatigue, and skin rashes. However, vasculitis often leads to more specific symptoms related to blood vessel inflammation, such as purpura (small purple spots on the skin), ulcers, or gangrene in severe cases. Lupus, on the other hand, is more likely to cause a butterfly-shaped rash on the face and sun sensitivity.

Blood tests in vasculitis may show elevated markers of inflammation, such as C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR), but these tests are not specific. A biopsy of the affected tissue, such as skin or kidney, can confirm vasculitis by showing blood vessel inflammation. In lupus, blood tests for ANA, anti-dsDNA, or anti-Smith antibodies are more commonly used to make a diagnosis, and organ involvement may be more systemic, particularly affecting the kidneys or heart.

Antiphospholipid Syndrome

Antiphospholipid syndrome (APS) is an autoimmune disorder that increases the risk of blood clots. People with APS may develop clots in the veins or arteries, leading to complications such as deep vein thrombosis (DVT), stroke, or pregnancy-related issues like recurrent miscarriages. APS can occur on its own or alongside other autoimmune diseases like lupus.

How to Know if You Might Have Antiphospholipid Syndrome vs. Systemic Lupus Erythematosus

Both APS and lupus can cause fatigue, joint pain, and other systemic symptoms, but APS is particularly associated with blood clotting issues. If you have had unexplained blood clots, recurrent miscarriages, or strokes at a young age, APS may be more likely. Lupus, while it can cause blood clotting problems, tends to have a broader range of symptoms, including skin rashes, kidney issues, and sensitivity to sunlight.

Blood tests for antiphospholipid antibodies (such as lupus anticoagulant, anti-cardiolipin, or anti-beta-2 glycoprotein I) are used to diagnose APS. In lupus, ANA, anti-dsDNA, or anti-Smith antibodies are more commonly tested. If you have both APS and lupus, you may test positive for both sets of antibodies.

Treatment Options for Systemic Lupus Erythematosus

Medications

Hydroxychloroquine

Hydroxychloroquine is an antimalarial drug commonly used to treat systemic lupus erythematosus (SLE). It works by modulating the immune system to reduce inflammation and prevent flare-ups.

Hydroxychloroquine is often prescribed as a first-line treatment for lupus and is typically used for long-term management. It is especially effective in controlling skin and joint symptoms and reducing the risk of lupus-related complications.

Patients can expect to see improvements in symptoms such as joint pain, skin rashes, and fatigue within several weeks to months of starting treatment. Long-term use can help prevent disease flares.

Corticosteroids

Corticosteroids are powerful anti-inflammatory drugs used to quickly reduce inflammation and suppress the immune system in lupus patients. Prednisone is a commonly prescribed corticosteroid.

Corticosteroids are typically used during lupus flare-ups or in cases of severe disease affecting vital organs like the kidneys or lungs. They are usually prescribed for short-term use due to their potential side effects, but low doses may be used for maintenance in some cases.

Patients often experience rapid relief from symptoms such as joint pain, swelling, and fatigue. However, long-term use can lead to side effects like weight gain, high blood pressure, and increased risk of infections.

Azathioprine

Azathioprine is an immunosuppressant that helps reduce the activity of the immune system, preventing it from attacking healthy tissues in lupus patients.

Azathioprine is often used in patients with moderate to severe lupus, particularly when corticosteroids alone are not sufficient or when long-term steroid use needs to be minimized. It may also be used in lupus nephritis (kidney involvement).

Patients may notice a gradual improvement in symptoms over several weeks to months. The goal is to reduce disease activity and prevent organ damage.

Methotrexate

Methotrexate is another immunosuppressant that reduces inflammation and suppresses the immune system. It is often used to treat lupus-related arthritis and skin issues.

Methotrexate is typically used in patients with mild to moderate lupus who have not responded well to other treatments like hydroxychloroquine or corticosteroids. It may also be used in combination with other medications.

Patients may experience a reduction in joint pain, swelling, and skin rashes within a few weeks to months of starting methotrexate. Regular monitoring is required due to potential side effects.

Belimumab

Belimumab is a biologic drug that targets a specific protein in the immune system called BLyS, which is involved in lupus-related inflammation.

Belimumab is typically used in patients with active lupus who have not responded well to standard treatments like corticosteroids or immunosuppressants. It is administered via injection or infusion.

Patients may notice a reduction in lupus flares and overall disease activity within several months of starting treatment. Belimumab can help improve quality of life by reducing the need for corticosteroids.

Rituximab

Rituximab is a biologic therapy that targets B-cells, a type of immune cell involved in lupus. By reducing the number of B-cells, rituximab helps lower inflammation and prevent further tissue damage.

Rituximab is usually reserved for patients with severe or refractory lupus, particularly those with lupus nephritis or central nervous system involvement. It is given as an intravenous infusion.

Patients may experience a reduction in disease activity within a few months of treatment. Rituximab can help prevent organ damage and reduce the need for high-dose corticosteroids.

Nonsteroidal Anti-inflammatory Drugs (NSAIDs)

NSAIDs, such as ibuprofen and naproxen, are commonly used to relieve pain and reduce inflammation in lupus patients.

NSAIDs are often used to manage mild symptoms like joint pain, muscle aches, and fever. They are typically used as a first-line treatment for mild lupus or in combination with other medications for more severe cases.

Patients can expect quick relief from pain and inflammation, usually within a few hours of taking NSAIDs. However, long-term use may lead to gastrointestinal side effects, so they should be used cautiously.

Cyclophosphamide

Cyclophosphamide is a potent immunosuppressant used to treat severe lupus, particularly when vital organs like the kidneys, heart, or lungs are affected.

Cyclophosphamide is typically reserved for patients with life-threatening lupus complications, such as lupus nephritis or central nervous system involvement. It is usually given as an intravenous infusion.

Patients may notice a reduction in disease activity within a few months of starting treatment. Cyclophosphamide can help prevent further organ damage, but it has significant side effects, including an increased risk of infections.

Mycophenolate Mofetil

Mycophenolate mofetil is an immunosuppressant that reduces the activity of the immune system and is often used to treat lupus nephritis.

Mycophenolate mofetil is typically used in patients with moderate to severe lupus, especially those with kidney involvement. It may be used as an alternative to cyclophosphamide in some cases.

Patients may experience a reduction in kidney inflammation and overall disease activity within several weeks to months. Regular monitoring is required to manage potential side effects.

Dapsone

Dapsone is an antibiotic with anti-inflammatory properties that is sometimes used to treat skin manifestations of lupus, such as rashes or sores.

Dapsone is typically used in patients with cutaneous (skin-related) lupus who have not responded well to other treatments like corticosteroids or hydroxychloroquine.

Patients may notice an improvement in skin symptoms within a few weeks of starting treatment. Dapsone is generally well-tolerated but requires regular blood tests to monitor for side effects.

Procedures

While medications are the cornerstone of lupus treatment, certain procedures may be necessary in severe cases or when specific organs are affected. These procedures are typically reserved for patients with advanced or life-threatening lupus complications.

Improving Systemic Lupus Erythematosus and Seeking Medical Help

In addition to medications, lifestyle changes and home remedies can play a significant role in managing lupus symptoms and improving overall well-being. Some helpful strategies include:

1. Sun protection: Wear sunscreen and protective clothing to prevent lupus-related skin flare-ups triggered by sun exposure.
2. Stress management: Practice relaxation techniques like deep breathing, meditation, or yoga to reduce stress, which can trigger lupus flares.
3. Regular exercise: Engage in low-impact activities like walking or swimming to improve joint flexibility and reduce fatigue.
4. Balanced diet: Eat a diet rich in fruits, vegetables, whole grains, and lean proteins to support overall health.
5. Adequate sleep: Ensure you get enough rest to help your body recover and reduce fatigue.
6. Avoid smoking: Smoking can worsen lupus symptoms and increase the risk of complications.
7. Limit alcohol intake: Excessive alcohol can interfere with medications and worsen lupus symptoms.
8. Stay hydrated: Drink plenty of water to support overall health and reduce fatigue.
9. Practice mindfulness: Mindfulness techniques can help manage pain and reduce the emotional toll of living with lupus.
10. Use supplements: Some patients find relief from symptoms by taking supplements like omega-3 fatty acids, but always consult your doctor before starting any new supplement.

Telemedicine offers a convenient way to manage lupus, allowing you to consult with healthcare providers from the comfort of your home. This is especially helpful for patients with mobility issues or those who need frequent monitoring. If you experience new or worsening symptoms, such as severe fatigue, joint pain, or signs of organ involvement, it’s important to seek medical help promptly.

Living with Systemic Lupus Erythematosus: Tips for Better Quality of Life

Living with lupus can be challenging, but there are several strategies to help improve your quality of life:

1. Follow your treatment plan closely and take medications as prescribed.
2. Stay active with low-impact exercises to keep your joints flexible and reduce fatigue.
3. Maintain a healthy diet and stay hydrated to support your overall health.
4. Get plenty of rest and manage stress to prevent flare-ups.
5. Stay in close communication with your healthcare provider, especially if you notice new or worsening symptoms.

Conclusion

Systemic lupus erythematosus is a complex autoimmune disease that can affect various parts of the body. Early diagnosis and treatment are crucial to managing symptoms and preventing complications. With a combination of medications, lifestyle changes, and regular monitoring, many patients can lead fulfilling lives despite their diagnosis.

If you suspect you may have lupus or are experiencing new symptoms, don’t hesitate to reach out to our primary care telemedicine practice. Our team of healthcare professionals is here to help you manage your condition and improve your quality of life.

James Kingsley

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