Signs and Symptoms of Systemic Lupus Erythematosus (SLE)
Systemic lupus erythematosus is a multisystem autoimmune disease primarily affecting women of reproductive age, characterized by cutaneous, musculoskeletal, renal, neurologic, hematological, and pulmonary manifestations that can lead to significant morbidity and mortality. 1
Common Clinical Manifestations
Constitutional Symptoms
- Fatigue (very common)
- Fever
- Weight loss
Mucocutaneous Manifestations
- Malar (butterfly) rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Alopecia
- Raynaud's phenomenon
Musculoskeletal Manifestations
- Arthritis (non-erosive)
- Arthralgia
- Myalgia
- Morning stiffness
Renal Manifestations
- Proteinuria
- Hematuria
- Cellular casts
- Elevated creatinine
Neuropsychiatric Manifestations
- Seizures (common, >5% cumulative incidence) 1
- Cerebrovascular disease (common, >5% cumulative incidence) 1
- Cognitive dysfunction (1-5% for severe form) 1
- Acute confusional state (1-5%) 1
- Psychosis (1-5%) 1
- Major depression (1-5%) 1
- Headache
Hematologic Manifestations
- Anemia (hemolytic or chronic disease)
- Leukopenia
- Lymphopenia
- Thrombocytopenia
Pulmonary Manifestations
- Pleural effusion (most common pulmonary manifestation) 1
- Dyspnea
- Chest pain
- Reduced exercise tolerance
- Cough
- Hemoptysis
- Interstitial lung disease (1-15% of patients) 1
Cardiovascular Manifestations
- Pericarditis
- Myocarditis
- Libman-Sacks endocarditis
- Accelerated atherosclerosis
Laboratory Findings
Immunologic Markers
- Positive ANA (>90% of patients) 2
- Anti-dsDNA antibodies
- Anti-Sm antibodies
- Low complement levels (C3, C4)
- Antiphospholipid antibodies (associated with cerebrovascular disease, seizures, and chorea) 1
- Anti-La/SSB, anti-Scl-70, and anti-U1RNP antibodies (associated with ILD development) 1
Other Laboratory Abnormalities
- Elevated inflammatory markers (ESR, CRP)
- Cytopenias (anemia, leukopenia, thrombocytopenia)
- Proteinuria/abnormal urinalysis
Disease Patterns and Timing
- Most neuropsychiatric events (50-60%) occur at disease onset or within the first year after SLE onset 1
- Between 20-90% of patients will develop respiratory involvement during the course of their disease 1
- About 40% of people with SLE develop lupus nephritis 3
Risk Factors for Specific Manifestations
For Interstitial Lung Disease
- Male gender
- Older age
- Advanced disease stage
- Previous episodes of acute lupus pneumonitis
- Raynaud phenomenon
- Gastroesophageal reflux disease
- Tachypnea
- Abnormal nail-fold capillaries
- Elevated CRP
- Anti-Sm and anti-U1-RNP seropositivity 1
For Neuropsychiatric Manifestations
- General SLE activity or damage
- Previous neuropsychiatric events
- Concurrent NPSLE manifestations
- Antiphospholipid antibodies 1
Clinical Pearls
- SLE symptoms often wax and wane, with periods of flares and remission 2
- Early manifestations typically include constitutional symptoms, mucocutaneous findings, and musculoskeletal complaints 4
- Diagnosis can be challenging as SLE mimics many other diseases 5
- The 2019 European Alliance of Associations for Rheumatology/American College of Rheumatology classification criteria are 96.1% sensitive and 93.4% specific for SLE 3
Early recognition of these signs and symptoms is crucial for timely diagnosis and management to prevent organ damage and improve patient outcomes.