Lupus (Systemic Lupus Erythematosus)

Systemic lupus erythematosus (SLE), commonly called lupus, is a complex autoimmune disease characterized by periods of illness (flares) and wellness (remission). In lupus, the immune system loses its ability to distinguish between foreign substances (antigens) and its own cells and tissues. This leads to the production of autoantibodies that attack the body's own tissues, causing inflammation, pain, and damage.

The word "lupus" means wolf in Latin, referring to the characteristic rash that was once thought to resemble a wolf's bite. "Systemic" means the disease can affect many organ systems, and "erythematosus" refers to the redness of the rash. While lupus can affect virtually any part of the body, each person's experience is unique – no two cases of lupus are exactly alike.

Lupus is not contagious, and you cannot "catch" it from someone or give it to someone else. The exact cause remains unknown, but researchers believe it results from a combination of genetic, environmental, and hormonal factors. While there is no cure for lupus, treatments have improved dramatically, allowing most people with lupus to live normal or near-normal lives with periods of remission.

Systemic Lupus Erythematosus (SLE)

• Most common and serious form (70% of cases)
• Can affect multiple organ systems
• Wide range of symptoms and severity
• What most people mean when they say "lupus"

Cutaneous Lupus

• Affects only the skin
• Three types: acute, subacute, and chronic (discoid)
• Can occur alone or with systemic lupus
• Discoid lupus causes circular, scarring rashes

Drug-Induced Lupus

• Caused by certain medications
• Symptoms similar to SLE but usually milder
• Goes away when medication is stopped
• Common culprits: hydralazine, procainamide, isoniazid

Neonatal Lupus

• Rare condition in newborns
• Caused by mother's antibodies
• Usually temporary (6 months)
• Can cause skin rash, liver problems, low blood counts
• Rarely causes permanent heart block

The Butterfly Rash

The malar or butterfly rash is one of lupus's most recognizable symptoms, occurring in about 40-50% of patients. It appears as a red or purple rash across the cheeks and bridge of the nose in a butterfly pattern. The rash may be flat or raised, can feel warm, and often worsens with sun exposure. However, many people with lupus never develop this rash.

Symptom Patterns

Lupus symptoms often come and go in patterns called flares and remissions. During a flare, symptoms worsen and new symptoms may appear. During remission, symptoms improve or disappear entirely. Flares can be triggered by stress, infections, sun exposure, certain medications, or hormonal changes.

Potential Causes

While the exact cause of lupus remains unknown, researchers believe multiple factors work together to trigger the disease:

Genetic Factors

• Having relatives with lupus increases risk
• Multiple genes likely involved
• Certain genetic markers increase susceptibility
• Not directly inherited like some genetic diseases

Environmental Triggers

• Ultraviolet light: Sun exposure can trigger flares
• Infections: Certain viruses may trigger lupus
• Medications: Some drugs can induce lupus-like symptoms
• Chemicals: Silica dust, cigarette smoke
• Physical or emotional stress: Can trigger flares

Hormonal Factors

• More common in women of childbearing age
• Estrogen may play a role
• Often appears or worsens during pregnancy
• Can flare with hormonal changes

Risk Factors

• Gender: Women are 9 times more likely to develop lupus
• Age: Usually develops between 15-45 years
• Race/Ethnicity: More common and severe in:
  • African Americans
  • Hispanics/Latinos
  • Asian Americans
  • Native Americans
  • Pacific Islanders
• Family history: 5-13% chance if first-degree relative has lupus

Diagnosing lupus can be challenging because symptoms vary widely and mimic many other conditions. No single test can diagnose lupus. Instead, doctors use a combination of medical history, physical examination, and laboratory tests.

Diagnostic Criteria

The American College of Rheumatology uses classification criteria for lupus. A person typically needs to meet at least 4 of 11 criteria, though diagnosis can be made with fewer if symptoms are highly suggestive:

1. Malar (butterfly) rash
2. Discoid rash
3. Photosensitivity
4. Oral or nasal ulcers
5. Non-erosive arthritis in 2+ joints
6. Pleuritis or pericarditis
7. Kidney disorder
8. Neurologic disorder
9. Blood disorder
10. Immunologic disorder
11. Positive ANA test

Laboratory Tests

Blood Tests

• ANA (Antinuclear Antibody): Positive in 95-98% of lupus patients
• Anti-dsDNA: Specific for lupus, indicates active disease
• Anti-Sm: Highly specific for lupus but only in 20-30%
• Anti-SSA/Ro and Anti-SSB/La: Associated with certain symptoms
• Complement levels (C3, C4): Low during active disease
• Complete blood count: May show anemia, low white cells, or low platelets
• ESR and CRP: Indicate inflammation

Other Tests

• Urinalysis: Check for kidney involvement
• 24-hour urine collection: Assess kidney function
• Chest X-ray: Look for lung or heart involvement
• Echocardiogram: Check heart function
• Kidney biopsy: If kidney involvement suspected
• Skin biopsy: For certain rashes

Treatment for lupus aims to prevent flares, treat symptoms when they occur, and minimize organ damage. The treatment plan depends on symptom severity and which organs are affected. Most people need a combination of medications.

Medications

Antimalarial Drugs

• Hydroxychloroquine (Plaquenil): Foundation of lupus treatment
• Helps with fatigue, joint pain, skin rashes
• May prevent flares and organ damage
• Generally well-tolerated with few side effects
• Requires regular eye exams

Corticosteroids

• Prednisone: Most common
• Powerful anti-inflammatory effects
• Used for moderate to severe symptoms
• Goal is lowest effective dose
• Long-term use has significant side effects

Immunosuppressants

• Mycophenolate mofetil (CellCept)
• Azathioprine (Imuran)
• Methotrexate
• Cyclophosphamide (Cytoxan) for severe cases
• Used for serious organ involvement

Biologics

• Belimumab (Benlysta): First drug approved specifically for lupus
• Rituximab: Used off-label for severe cases
• Target specific parts of immune system
• Given by injection or infusion

Other Medications

• NSAIDs: For joint pain and fever
• Blood pressure medications: Protect kidneys
• Anticoagulants: If blood clotting issues
• Bone-strengthening drugs: If on long-term steroids

Lifestyle Management

• Sun protection: Sunscreen, protective clothing, avoid peak hours
• Regular exercise: Helps with fatigue and joint stiffness
• Healthy diet: Anti-inflammatory foods, limit salt if kidney issues
• Stress management: Meditation, counseling, support groups
• Adequate rest: Balance activity with rest
• No smoking: Smoking worsens lupus
• Limit alcohol: Can interact with medications

Kidney Complications (Lupus Nephritis)

• Occurs in about 50% of lupus patients
• Can lead to kidney failure if untreated
• May require dialysis or transplant
• Regular monitoring essential

Cardiovascular Complications

• Increased risk of heart disease
• Pericarditis (inflammation of heart lining)
• Myocarditis (heart muscle inflammation)
• Increased risk of stroke
• Blood clots (especially with antiphospholipid antibodies)

Neurological Complications

• Headaches and migraines
• Seizures
• Cognitive dysfunction ("lupus fog")
• Mood disorders
• Peripheral neuropathy
• Stroke

Blood and Immune System

• Anemia
• Increased bleeding or clotting
• Increased infection risk
• Lymphoma risk slightly increased

Pregnancy Complications

• Increased risk of miscarriage
• Preterm birth
• Preeclampsia
• Neonatal lupus in baby
• Requires specialized care

Other Complications

• Avascular necrosis (bone death from reduced blood flow)
• Osteoporosis (from steroids)
• Increased cancer risk
• Eye problems

Daily Management

• Take medications as prescribed: Even when feeling well
• Track symptoms: Keep a diary of symptoms and triggers
• Regular medical care: Keep all appointments
• Monitor for warning signs: Know when to call doctor
• Protect from sun: Daily sunscreen, protective clothing
• Stay active: Regular, gentle exercise
• Eat well: Balanced, anti-inflammatory diet

Managing Flares

• Learn your warning signs
• Contact doctor early in flare
• Rest more during flares
• Avoid known triggers
• May need medication adjustments
• Don't stop medications without consulting doctor

Work and School

• Know your rights under disability laws
• Consider workplace accommodations
• Flexible scheduling may help
• Communicate with employers/teachers
• Plan for days when symptoms worsen

Emotional Health

• Depression and anxiety are common
• Seek mental health support
• Join support groups
• Online communities can help
• Family counseling may be beneficial

Family Planning

• Pregnancy is possible but requires planning
• Best outcomes when disease is stable
• Some medications must be stopped
• Work with high-risk pregnancy specialist
• Discuss contraception with doctor

Common Triggers to Avoid

• Sun exposure: Use SPF 30+, wear protective clothing
• Stress: Practice stress management techniques
• Infections: Stay up-to-date on vaccines, practice good hygiene
• Certain medications: Discuss all new medications with doctor
• Overexertion: Balance activity with rest
• Poor sleep: Maintain good sleep hygiene
• Smoking: Quit if you smoke

Preventive Care

• Regular check-ups with rheumatologist
• Annual flu vaccine (avoid live vaccines)
• Pneumonia vaccine
• Regular cancer screenings
• Bone density tests if on steroids
• Eye exams if on Plaquenil
• Cardiovascular risk assessment

The prognosis for people with lupus has improved dramatically over the past several decades. In the 1950s, most people with lupus lived less than 5 years after diagnosis. Today, with early diagnosis and proper treatment:

• 85-90% of people with lupus live more than 10 years after diagnosis
• Many people have a normal or near-normal lifespan
• Most can work, have families, and lead fulfilling lives
• Quality of life continues to improve with new treatments

Factors Affecting Prognosis

• Early diagnosis and treatment: Better outcomes
• Organ involvement: Kidney, heart, brain involvement more serious
• Access to care: Regular monitoring improves outcomes
• Treatment adherence: Taking medications as prescribed
• Lifestyle factors: Not smoking, sun protection, healthy habits