Signs and Symptoms of Systemic Lupus Erythematosus (SLE)
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease primarily affecting women of reproductive age, characterized by a wide range of clinical manifestations including cutaneous, musculoskeletal, renal, neurologic, hematological, and pulmonary symptoms. 1
Common Clinical Manifestations
Constitutional Symptoms
- Fatigue (often severe and persistent)
- Fever (typically low-grade)
- Weight loss
- General malaise
Mucocutaneous Manifestations
- Skin involvement:
- Butterfly (malar) rash across cheeks and nose
- Discoid lesions (scarring, disk-shaped lesions)
- Photosensitivity (skin rash after sun exposure)
- Subacute cutaneous lupus (non-scarring, photosensitive rash)
- Mucosal involvement:
- Oral/nasal ulcers (typically painless)
- Hair:
- Alopecia (hair loss, often patchy)
- Raynaud's phenomenon (fingers turning white/blue in cold)
Musculoskeletal Manifestations
- Arthritis (non-erosive, affecting multiple joints)
- Arthralgia (joint pain without inflammation)
- Myalgia (muscle pain)
- Myositis (muscle inflammation)
- Avascular necrosis (especially with steroid use)
Cardiovascular Manifestations
- Pericarditis
- Myocarditis
- Libman-Sacks endocarditis
- Accelerated atherosclerosis
- Coronary artery disease (increased risk)
Pulmonary Manifestations
- Pleuritis (most common pulmonary manifestation)
- Pleural effusion
- Interstitial lung disease (in 1-15% of patients)
- Acute lupus pneumonitis
- Diffuse alveolar hemorrhage (rare but life-threatening)
- Pulmonary hypertension
Renal Manifestations
- Lupus nephritis (affects approximately 40% of patients)
- Proteinuria
- Hematuria
- Cellular casts in urine
- Elevated serum creatinine
Neuropsychiatric Manifestations
- Seizures
- Psychosis
- Cognitive dysfunction
- Headache
- Peripheral neuropathy
- Cranial neuropathy
- Acute confusional state
- Transverse myelitis
Hematologic Manifestations
- Anemia (often hemolytic)
- Leukopenia
- Lymphopenia
- Thrombocytopenia
Gastrointestinal Manifestations
- Peritonitis
- Mesenteric vasculitis
- Pancreatitis
- Hepatitis
Laboratory Findings
Immunologic Abnormalities
- Positive ANA (antinuclear antibody) - present in >95% of patients
- Anti-dsDNA antibodies
- Anti-Sm antibodies
- Anti-Ro/SSA antibodies
- Anti-La/SSB antibodies
- Anti-RNP antibodies
- Antiphospholipid antibodies
- Low complement levels (C3, C4)
- Anti-C1q antibodies
Other Laboratory Findings
- Elevated inflammatory markers (ESR, CRP)
- Cytopenias (anemia, leukopenia, thrombocytopenia)
- Proteinuria and/or hematuria
- Elevated serum creatinine
Disease Patterns and Monitoring
SLE typically follows a relapsing-remitting course with periods of flares and remissions. The clinical presentation can vary significantly between patients, with some experiencing predominantly skin and joint symptoms while others develop severe organ involvement 1.
Risk Factors for Poor Prognosis
- Male sex
- Younger age at onset
- African or Hispanic ethnicity
- Low socioeconomic status
- Presence of certain antibodies (anti-dsDNA, anti-Sm, anti-RNP)
- Major organ involvement (renal, neuropsychiatric, cardiopulmonary)
- Persistent disease activity
- Early damage accrual
Diagnostic Approach
While there are no universally accepted diagnostic criteria for SLE, the 2019 EULAR/ACR classification criteria are commonly used, which include both clinical and immunological factors 2.
Clinical Domains
- Constitutional symptoms
- Hematologic abnormalities
- Neuropsychiatric manifestations
- Mucocutaneous lesions
- Serosal involvement
- Musculoskeletal manifestations
- Renal involvement
Immunological Domains
- Antinuclear antibodies
- Anti-dsDNA antibodies
- Anti-Sm antibodies
- Antiphospholipid antibodies
- Low complement levels
- Direct Coombs test
Common Pitfalls in Diagnosis
- Delayed diagnosis due to the heterogeneous presentation and overlap with other conditions
- Overdiagnosis based solely on positive ANA without supporting clinical features
- Failure to recognize organ-threatening manifestations requiring urgent treatment
- Attributing all symptoms to SLE without considering comorbidities or medication side effects
- Inadequate monitoring of disease activity and organ involvement
Early recognition of SLE signs and symptoms is crucial for timely diagnosis and treatment to prevent organ damage and improve long-term outcomes.