Critical Alerts

• Distinguish flare from infection - both can present similarly
• Lupus nephritis can progress rapidly to renal failure
• CNS lupus is life-threatening and requires aggressive treatment
• Catastrophic antiphospholipid syndrome has high mortality
• Patients on immunosuppression are at high infection risk

Key Diagnostics

• CBC (cytopenias common in flare)
• BMP (renal function, electrolytes)
• Urinalysis with microscopy (active sediment in nephritis)
• Anti-dsDNA, complement levels (C3, C4)
• ESR, CRP (CRP often normal in flare unless infection)
• Cultures (blood, urine) to exclude infection

Emergency Treatments

• Methylprednisolone: 1g IV daily x 3 days for severe flare
• Continue/increase baseline immunosuppression: Per rheumatology
• Hydroxychloroquine: Continue unless toxic
• Treat infection if present: Broad-spectrum antibiotics
• Dialysis: For severe renal failure

Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease characterized by autoantibody production and immune complex deposition, causing inflammation and damage to multiple organ systems. A flare is defined as a measurable increase in disease activity requiring therapeutic intervention.

Disease Manifestations

Epidemiology

• Prevalence: 50-100 per 100,000
• Sex ratio: Female:Male 9:1
• Peak onset: 15-45 years
• Higher incidence: African American, Hispanic, Asian populations
• Mortality: 5-year survival >90% but reduced compared to general population

Flare Triggers

Critical Presentations

Catastrophic Antiphospholipid Syndrome (CAPS)

Definition: Multi-organ thrombosis over days to weeks

Features:

• Renal failure
• ARDS
• Cardiac involvement
• CNS involvement
• DIC-like picture

Treatment: Anticoagulation + steroids + plasma exchange + IVIG

Flare vs Infection

Other Conditions

Initial Assessment

Key History

• Known SLE diagnosis and baseline disease activity
• Current medications and adherence
• Recent triggers (sun exposure, infection, stress)
• Symptoms suggesting organ involvement
• Any recent medication changes

Laboratory Studies

Imaging

Special Studies

ICU Admission Criteria

• Altered mental status/CNS lupus
• Diffuse alveolar hemorrhage
• Severe respiratory distress
• Cardiovascular instability
• Catastrophic APS
• Dialysis requirement
• Life-threatening cytopenias

Hospital Ward Admission

• Moderate-severe flare requiring IV steroids
• Lupus nephritis (new or significantly worsening)
• Need for infection workup
• Inability to take oral medications
• Close monitoring needed

Discharge Criteria (Mild Flare)

• Stable, mild disease activity
• No organ-threatening features
• Able to take oral medications
• Reliable follow-up with rheumatology
• Understands warning signs

Follow-up Recommendations

Understanding SLE Flares

• Lupus is a chronic condition with periods of activity (flares) and remission
• Flares can range from mild to life-threatening
• Early treatment of flares prevents organ damage
• Medication adherence is crucial

Recognizing Flare Symptoms

• Increased fatigue
• New or worsening rash
• Joint pain or swelling
• Fever not explained by infection
• Chest pain (breathing-related)
• Swelling of face or legs
• Blood in urine

When to Seek Emergency Care

• Chest pain or difficulty breathing
• Severe headache or vision changes
• Confusion or seizures
• Coughing blood
• Severe abdominal pain
• Fever with new symptoms on immunosuppression

Lifestyle Measures

• Strict sun protection (SPF 50+, protective clothing)
• Medication adherence
• Regular follow-up
• Vaccinations (inactivated vaccines safe)
• Smoking cessation
• Stress management

Pregnancy

• Pregnancy can trigger flares
• Lupus nephritis worsens outcomes
• Antiphospholipid syndrome increases pregnancy loss
• Certain medications contraindicated (mycophenolate, cyclophosphamide)
• High-risk obstetric care needed
• Continue hydroxychloroquine (reduces flares)

Pediatric SLE

• More aggressive disease than adult-onset
• Higher rates of nephritis
• More commonly involves CNS
• Long-term complications from steroids

End-Stage Renal Disease

• May develop from nephritis
• Hemodialysis or transplant options
• Disease activity often decreases with ESRD
• Immunosuppression considerations

Elderly-Onset SLE

• Less common
• May have different presentation
• More drug-induced lupus
• Similar treatment principles

Performance Indicators

Documentation Requirements

• Current SLE medications
• Baseline disease activity
• New symptoms and organ involvement
• Laboratory results including complement
• Infection evaluation
• Treatment provided
• Follow-up plan
• Rheumatology communication

Diagnostic Pearls

1. CRP normal in pure flare - if elevated, think infection
2. Complement low + anti-dsDNA high = active lupus
3. Lymphopenia is common and doesn't indicate infection
4. Active urine sediment (RBC casts) = nephritis
5. Don't forget APS - can cause thrombosis during flare

Treatment Pearls

1. Pulse steroids for severe flare - 1g methylprednisolone x 3 days
2. Don't stop hydroxychloroquine - it prevents flares
3. Treat infection AND flare if both present
4. Early rheumatology involvement improves outcomes
5. Monitor for steroid side effects - infection, glucose, BP

Disposition Pearls

1. Low threshold to admit if any organ involvement
2. ICU for CNS lupus, DAH, CAPS
3. Nephrology for lupus nephritis
4. Close follow-up essential - disease can progress rapidly
5. Patient education on warning signs is critical