Signs and Symptoms of Systemic Lupus Erythematosus (SLE)
Systemic lupus erythematosus is a multisystem autoimmune disease characterized by cutaneous, musculoskeletal, renal, neurologic, hematological, and pulmonary manifestations, primarily affecting women of reproductive age. 1
Common Clinical Manifestations
Constitutional Symptoms
- Fatigue
- Fever
- Weight loss
Mucocutaneous Manifestations (Common)
- Acute cutaneous lupus (malar/butterfly rash)
- Subacute cutaneous lupus (photosensitive, non-scarring lesions)
- Chronic cutaneous lupus (discoid lesions with scarring)
- Oral/nasal ulcers
- Alopecia
- Photosensitivity
Musculoskeletal Manifestations (Common)
- Arthritis/arthralgia (non-erosive, involving small joints of hands, wrists, knees)
- Myalgia
- Morning stiffness
Renal Manifestations
- Proteinuria
- Hematuria
- Cellular casts
- Elevated serum creatinine
- Hypertension
Neuropsychiatric Manifestations
- Common (>5% incidence) 1:
- Cerebrovascular disease
- Seizures
- Less common (1-5% incidence):
- Severe cognitive dysfunction
- Major depression
- Acute confusional state
- Peripheral neuropathy
- Psychosis
Hematological Manifestations
- Leukopenia
- Lymphopenia
- Thrombocytopenia
- Hemolytic anemia
Cardiopulmonary Manifestations
- Pleuritis (most common pulmonary manifestation) 1
- Pericarditis
- Myocarditis
- Interstitial lung disease (rare, 1-15% of patients) 1
- Pulmonary hypertension
Risk Factors for Specific Manifestations
Neuropsychiatric Lupus Risk Factors 1
- Previous or concurrent severe NPSLE (for cognitive dysfunction, seizures)
- Antiphospholipid antibodies (for cerebrovascular disease, seizures, chorea)
- General SLE activity or damage
Interstitial Lung Disease Risk Factors 1
- Male gender
- Older age
- Advanced disease stage
- Previous acute lupus pneumonitis
- Raynaud phenomenon
- Gastroesophageal reflux disease
- Tachypnea
- Abnormal nail-fold capillaries
- Elevated CRP
- Anti-Sm and anti-U1-RNP seropositivity
Diagnostic Considerations
Laboratory Findings
- Positive ANA (antinuclear antibodies)
- Anti-dsDNA antibodies
- Anti-Sm antibodies
- Low complement levels (C3, C4)
- Antiphospholipid antibodies
- Elevated inflammatory markers (ESR)
- CRP may be normal or mildly elevated (significant elevation suggests infection) 1
Diagnostic Workup for Specific Manifestations
Neuropsychiatric Manifestations 1
- CSF analysis (to exclude CNS infection)
- EEG (for seizure disorders)
- Neuropsychological tests (for cognitive dysfunction)
- MRI (T1/T2, fluid-attenuating inversion recovery, diffusion-weighted imaging)
Pulmonary Manifestations 1
- Pulmonary function tests (spirometry, DLCO)
- Chest radiography
- HRCT for suspected interstitial lung disease
Renal Manifestations 2
- Urinalysis
- Proteinuria quantification
- Renal function tests
- Renal biopsy (when indicated)
Monitoring Considerations
General Monitoring 2
- Complete blood count every 1-3 months during active disease
- Anti-dsDNA antibodies and complement levels (C3, C4)
- Renal function tests and urinalysis
Organ-Specific Monitoring
- Cutaneous: Consider using Cutaneous Lupus Disease Area and Severity Index (CLASI) 1
- Renal: Regular monitoring of serum creatinine, urinalysis, proteinuria, and blood pressure 1
- Neuropsychiatric: Clinical history and cognitive assessment 1
- Ophthalmologic: Baseline and periodic eye exams for patients on antimalarials 1, 2
Important Clinical Pitfalls
- Symptoms may fluctuate and vary widely between patients
- Diagnosis can be delayed due to nonspecific early symptoms
- Neuropsychiatric manifestations may be mistaken for primary psychiatric disorders
- Infections can mimic lupus flares and should be excluded
- CRP elevation >50 mg/L suggests infection rather than lupus activity 1
- Medication side effects may mimic disease manifestations
Remember that SLE is a complex disease with variable presentation, and early recognition of signs and symptoms is crucial for timely diagnosis and management to prevent organ damage and improve outcomes.