What are the symptoms and treatment options for a lupus flare in a young to middle-aged woman with a history of lupus?

Symptoms of Lupus Flare

Lupus flares present with a constellation of constitutional, mucocutaneous, musculoskeletal, hematologic, renal, and neuropsychiatric symptoms that require immediate recognition and severity stratification to guide treatment intensity. 1

Constitutional and General Symptoms

• Fever is a common presenting symptom of lupus flare, though significantly elevated C-reactive protein (>50 mg/L) should prompt evaluation for superimposed infection rather than lupus activity alone 2
• Fatigue represents one of the earliest and most frequent complaints during disease exacerbation 3
• Weight changes and general malaise accompany active disease 1

Mucocutaneous Manifestations

• Lupus-specific skin lesions include acute cutaneous lupus (malar rash, photosensitive rash), subacute cutaneous lupus, chronic cutaneous lupus (discoid lesions), and intermittent cutaneous lupus 2
• Mouth ulcers (oral or nasopharyngeal ulceration) occur frequently during flares 3
• Alopecia (non-scarring hair loss) develops with active disease 3
• Purpura, petechial rash, and necrotic-like skin lesions may appear in severe cases 4
• Skin manifestations require evaluation by an experienced dermatologist, as many conditions mimic lupus and biopsy may be necessary for definitive diagnosis 2

Musculoskeletal Symptoms

• Arthritis affecting multiple joints is characteristic of mild-to-moderate flares 1
• Joint pain and myalgia represent early complaints in most patients 3
• Psoriatic arthritis without skin psoriasis can occur in some lupus patients 4

Renal Involvement (Lupus Nephritis)

• Proteinuria (elevated urine protein:creatinine ratio) indicates active kidney involvement and predicts flare occurrence 2
• Rising serum creatinine and declining estimated glomerular filtration rate signal worsening renal function 2
• Active urine sediment with cellular casts suggests active nephritis 2
• Hypertension develops or worsens with renal flares 2
• Edema (particularly eyelid edema) may accompany nephrotic-range proteinuria 4
• Renal flares occur in up to 45% of lupus nephritis patients, with a flare rate of 0.1-0.2 flares/patient/year 2

Hematologic Abnormalities

• Severe anemia associates with organ involvement and disease progression 2
• Thrombocytopenia correlates with renal disease and worse prognosis 2
• Severe leukopenia and lymphopenia increase infection risk and indicate active disease 2
• Cytopenias (low blood counts) characterize severe flares 1

Neuropsychiatric Manifestations

• Headache is the most frequent neurological symptom 2
• Mood disorders (depression, anxiety) occur commonly 2
• Seizures indicate severe central nervous system involvement 2, 1
• Cognitive impairment manifests as memory problems, attention deficits, concentration difficulties, and word-finding problems 2
• Cerebrovascular disease (stroke, transient ischemic attacks) represents severe organ-threatening involvement 2
• Neuropsychiatric symptoms require careful assessment through clinical history, as no specific validated instrument exists for routine practice 2

Cardiopulmonary Involvement

• Serositis (pleuritis, pericarditis) causes chest pain and may be mild-to-moderate or severe depending on extent 1
• Pulmonary infiltrates with hypoxemia may indicate lupus pneumonitis, though infection must be excluded first 5
• Cardiopulmonary involvement in severe flares is organ-threatening and requires aggressive treatment 1

Vascular Manifestations

• Vasculitis represents severe, organ-threatening disease requiring immediate intervention 1
• Thrombotic events may occur, particularly in patients with antiphospholipid antibodies 2

Laboratory Markers of Flare Activity

• Rising anti-dsDNA antibody levels correlate with disease activity, particularly renal flares 2
• Low complement levels (C3, C4) indicate active disease and immune complex consumption 2
• Hypoalbuminemia suggests renal involvement with protein loss 2
• These markers should be monitored at each visit during active disease 2

Severity Stratification

Mild-to-moderate flares include constitutional symptoms, arthritis, rash, or mild serositis without major organ involvement 1

Severe/organ-threatening flares encompass active lupus nephritis, neuropsychiatric manifestations, severe cytopenias, cardiopulmonary involvement, or vasculitis 1

Critical Pitfall to Avoid

Never escalate immunosuppression in a febrile lupus patient with new pulmonary infiltrates without first excluding infection through bronchoscopy with bronchoalveolar lavage, as this represents a potentially fatal error 5. Fever with significantly elevated CRP (>50 mg/L) should trigger infection workup rather than assumption of pure lupus activity 2.