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Systemic Lupus Erythematosus (SLE) Explained

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease in which the immune system mistakenly attacks healthy tissue, potentially affecting multiple organs throughout the body.

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Things worth knowing about "Systemic lupus erythematosus"

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease in which the immune system mistakenly attacks healthy tissue, potentially affecting multiple organs throughout the body.

What is systemic lupus erythematosus?

Systemic lupus erythematosus (SLE) is a chronic, inflammatory autoimmune disease in which the immune system mistakenly targets the body's own healthy tissues. The term “systemic” reflects the fact that virtually any organ or organ system can be involved, including the skin, joints, kidneys, heart, lungs, blood vessels, and nervous system. SLE follows a relapsing-remitting course and predominantly affects women of childbearing age.

Causes

The exact cause of SLE has not been fully established. It is considered a multifactorial condition in which genetic, hormonal, and environmental factors interact.

Genetic factors: Certain gene variants increase susceptibility to SLE. First-degree relatives of affected individuals carry a higher risk.
Hormonal factors: Estrogen appears to play a role, as women are significantly more commonly affected than men.
Environmental triggers: UV radiation, infections (e.g., Epstein-Barr virus), certain medications (drug-induced lupus), and psychological stress can precipitate flares.
Immunological dysregulation: Defective clearance of cellular debris leads to the production of autoantibodies, particularly antinuclear antibodies (ANA) and anti-double-stranded DNA (anti-dsDNA) antibodies.

Symptoms

SLE presents with a wide spectrum of symptoms that vary considerably from person to person. Common manifestations include:

Butterfly rash (malar rash): A characteristic butterfly-shaped rash across the nose and cheeks.
Joint and muscle pain: Arthritis and myalgia, often symmetrical and migratory.
Fatigue: Profound exhaustion that significantly impairs quality of life.
Photosensitivity: Increased sensitivity to sunlight with skin reactions following UV exposure.
Hematological changes: Anemia, leukopenia, or thrombocytopenia.
Lupus nephritis: Kidney involvement with proteinuria and possible renal insufficiency.
Neurological symptoms: Headaches, seizures, and cognitive impairment.
Serositis: Inflammation of the lining around the heart or lungs (pericarditis, pleuritis).
Raynaud's phenomenon: Reduced blood flow to the fingers in response to cold.

Diagnosis

The diagnosis of SLE is based on a combination of clinical findings and laboratory investigations. Standardized classification criteria assist clinicians in making an accurate diagnosis.

Antinuclear antibodies (ANA): Positive in more than 95% of SLE patients, though not specific to SLE alone.
Anti-dsDNA antibodies: Highly specific for SLE and often correlate with disease activity.
Anti-Sm antibodies: Also highly specific for SLE.
Complement levels (C3, C4): Reduced levels may indicate active disease.
Complete blood count, renal function tests, urinalysis: To assess potential organ involvement.
Imaging and biopsy: Used when renal, cardiac, or pulmonary involvement is suspected.

The EULAR/ACR 2019 classification criteria serve as an important reference for diagnosing SLE.

Treatment

There is currently no cure for SLE. The goals of treatment are to control disease activity, prevent organ damage, and improve quality of life.

Pharmacological treatment

Antimalarial drugs (hydroxychloroquine): The cornerstone of treatment for nearly all SLE patients; reduces flares and long-term organ damage.
Corticosteroids (e.g., prednisolone): Used for rapid suppression of inflammation; the dose should be kept as low as possible and for the shortest duration necessary.
Immunosuppressants: Azathioprine, mycophenolate mofetil, or methotrexate for moderate to severe disease.
Biologics: Belimumab (anti-BLyS antibody) and anifrolumab (anti-interferon receptor antibody) are approved biological therapies for SLE.
Cyclophosphamide: Reserved for severe organ involvement, particularly lupus nephritis.

General measures

Sun protection (high-factor sunscreen, protective clothing)
Regular medical check-ups and laboratory monitoring
Optimizing vaccination status (live vaccines are contraindicated during immunosuppression)
Healthy lifestyle: balanced diet, moderate physical activity, stress management

Prognosis

The prognosis of SLE has improved considerably with modern treatment options. Many patients lead largely normal lives. Long-term outcomes depend significantly on the extent of organ involvement, particularly of the kidneys. Regular follow-up and consistent adherence to therapy are essential.

References

Fanouriakis A et al. - 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Annals of the Rheumatic Diseases, 2019; 78(6): 736-745.
Aringer M et al. - 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis & Rheumatology, 2019; 71(9): 1400-1412.
Tsokos GC - Systemic Lupus Erythematosus. New England Journal of Medicine, 2011; 365(22): 2110-2121.

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