Lupus (systemic lupus erythematosus)

Systemic lupus erythematosus (SLE, or lupus) is a chronic autoimmune disease in which the immune system attacks its own tissues, causing inflammation and damage that can affect the joints, skin, kidneys, blood cells, brain, heart, and lungs.

Lupus is more common in women, especially during childbearing years, and is more frequent and often more severe in people of African, Hispanic, Asian, and Native American ancestry. The course is unpredictable, with flares and remissions. There is no cure, but treatment can control symptoms, reduce flares, and limit organ damage. Many people with lupus lead full lives with appropriate care.

Symptoms vary widely and can change over time. Common features include:

• Fatigue and fever
• Joint pain, stiffness, and swelling
• Butterfly-shaped rash on the face (malar rash) that covers the cheeks and bridge of the nose
• Skin lesions that worsen with sun exposure
• Raynaud’s phenomenon (fingers and toes turn white or blue with cold or stress)
• Shortness of breath or chest pain (pleuritis or pericarditis)
• Dry eyes
• Headaches, confusion, or memory difficulties
• Mouth ulcers
• Hair loss
• Swollen glands
• Swelling in the legs or around the eyes (possible kidney involvement)

No two cases are exactly alike. Mild disease may involve mainly skin and joints; severe disease can threaten major organs.

Lupus results from a complex interaction of genetic susceptibility and environmental triggers that lead to loss of immune tolerance and production of autoantibodies (including antinuclear antibodies). Triggers that may provoke onset or flares in predisposed people include:

• Sunlight / ultraviolet exposure
• Infections
• Certain medicines (drug-induced lupus is a related but usually reversible condition)
• Stress
• Hormonal factors (including estrogen)

It is not contagious.

Risk factors include:

• Female sex
• Age 15–45 at onset (though lupus can occur in children and older adults)
• Family history of lupus or other autoimmune disease
• Race/ethnicity — higher incidence and often greater severity in African American, Hispanic, Asian, and Native American populations
• Other autoimmune diseases in the individual

Lupus can affect nearly any organ. Important complications include:

• Lupus nephritis and chronic kidney disease
• Cardiovascular disease (accelerated atherosclerosis, pericarditis, myocarditis)
• Neurologic and psychiatric involvement (seizures, stroke, cognitive dysfunction, mood disorders)
• Blood disorders (anemia, thrombocytopenia, leukopenia, antiphospholipid antibodies with clotting risk)
• Lung disease (pleuritis, pneumonitis, pulmonary hypertension)
• Increased infection risk from both the disease and immunosuppressive treatment
• Pregnancy complications (including preeclampsia and neonatal lupus)
• Osteoporosis and avascular necrosis (partly related to corticosteroids)

Lupus cannot be prevented. Once diagnosed, steps that help prevent flares and organ damage include:

• Strict sun protection (sunscreen, protective clothing, avoiding peak UV hours)
• Not smoking
• Adhering to prescribed medicines
• Prompt treatment of infections
• Regular monitoring of blood pressure, kidney function, and other organ parameters
• Vaccinations as recommended (avoid live vaccines when heavily immunosuppressed)
• Pregnancy planning with rheumatology and high-risk obstetrics

Diagnosis is based on a combination of clinical findings and laboratory tests. There is no single definitive test. Evaluation typically includes:

• Detailed history and physical examination
• Antinuclear antibody (ANA) test — sensitive but not specific
• Specific autoantibodies (anti-dsDNA, anti-Smith, anti-Ro/La, antiphospholipid antibodies, etc.)
• Complement levels (C3, C4)
• Complete blood count, chemistry panel, urinalysis
• Classification criteria (EULAR/ACR) used as a guide, not a strict checklist
• Imaging or organ-specific tests when involvement is suspected

Diagnosis can take time because symptoms often appear gradually and overlap with other conditions.

See a healthcare professional for an unexplained rash, ongoing fever, persistent joint pain or swelling, or extreme fatigue. Seek urgent care for chest pain, severe shortness of breath, significant swelling, severe headache, confusion, or markedly reduced urine output.

People already diagnosed with lupus should contact their care team promptly when they notice signs of a flare or new organ symptoms.

Treatment is individualized according to disease severity and organ involvement:

• Hydroxychloroquine — backbone therapy for most patients; reduces flares and improves long-term outcomes
• NSAIDs and short courses of corticosteroids — for mild musculoskeletal or serosal symptoms
• Immunosuppressants — methotrexate, azathioprine, mycophenolate, cyclophosphamide for more serious disease
• Biologics — belimumab, anifrolumab, and others in selected patients
• Voclosporin and other agents for lupus nephritis in combination regimens
• Anticoagulation when antiphospholipid syndrome is present
• Supportive care — blood-pressure control, bone protection, infection prevention, mental-health support

The goal is remission or low disease activity with the least toxicity possible.

Daily self-management includes:

• Taking medicines consistently and attending laboratory monitoring
• Sun protection every day
• Not smoking
• Regular, moderate exercise as tolerated
• Adequate rest and pacing during flares
• Stress management and mental-health support
• Prompt reporting of fever, new rashes, swelling, chest pain, or neurologic changes
• Working closely with a rheumatologist and other specialists as needed

Bring:

• List of all symptoms and when they started
• Photos of rashes if they come and go
• Personal and family history of autoimmune disease
• Complete medication list
• Previous laboratory and biopsy results
• Questions about treatment goals, pregnancy, sun protection, and monitoring