What are the clinical features of Systemic Lupus Erythematosus (SLE)?

Clinical Features of Systemic Lupus Erythematosus

Systemic lupus erythematosus presents with an extremely variable clinical picture that may involve multiple organ systems, with constitutional, mucocutaneous, and musculoskeletal symptoms representing the earliest and most common manifestations. 1, 2

Constitutional and General Symptoms

• Fatigue is one of the earliest and most common complaints in SLE patients 2
• Fever may occur and is included in classification criteria as a clinical indicator 3
• Quality of life is significantly impacted by disease activity, organ damage, and drug toxicity 1

Mucocutaneous Manifestations

• Lupus-specific rash is a hallmark early presentation, with approximately 88% of patients having mucocutaneous involvement at baseline 4, 2
• Photosensitive cutaneous disease is particularly common, especially in patients with anti-Ro/SSA antibodies 5
• Mouth ulcers (oral or nasopharyngeal ulceration) are characteristic features 2
• Alopecia (hair loss) occurs frequently 2
• Discoid lupus lesions may develop, requiring specific management with topical corticosteroids and hydroxychloroquine 6

Musculoskeletal Features

• Joint pain (arthralgia) and arthritis are among the earliest complaints, with approximately 78% of patients having musculoskeletal involvement at baseline 4, 2
• Myalgia (muscle pain) is common 2
• Non-erosive arthritis affecting multiple joints is typical 3

Renal Involvement

• Lupus nephritis develops in approximately 40% of people with SLE 3
• Proteinuria is a key indicator of renal involvement and included in classification criteria 3
• Approximately 10% of patients with lupus nephritis progress to end-stage kidney disease after 10 years 3
• Renal involvement may be asymptomatic initially, requiring regular monitoring with urinalysis, urine protein-to-creatinine ratio, and renal function tests 5

Hematologic Abnormalities

• Cytopenia (low blood cell counts) is a common feature included in classification criteria 3
• Immunologic involvement occurs in approximately 76% of patients at baseline 4
• Autoantibody production is characteristic, including anti-double-stranded DNA, anti-Sm, anti-Ro/SSA, and anti-La/SSB antibodies 3, 2
• Hypocomplementemia (low C3 and/or C4 levels) reflects active disease 2

Cardiovascular Manifestations

• Serositis (pleuritis or pericarditis) may occur 3
• Patients have markedly increased risk of premature atherosclerosis and cardiovascular disease that cannot be fully explained by traditional risk factors 1
• Hypertension prevalence ranges from 11.5% to 75% 1
• Dyslipidemia prevalence ranges from 11.5% to 75% 1
• Vascular involvement occurs in less than 15% of patients but is clinically significant 4

Neuropsychiatric Manifestations

• Neuropsychiatric lupus can present with diverse manifestations including psychosis, seizures, acute confusional state, cranial or peripheral neuropathy, and transverse myelitis 1
• Less than 15% of patients have CNS system activity at baseline, but these manifestations significantly impact morbidity 4
• Diagnostic work-up should be similar to that in the general population presenting with the same neuropsychiatric symptoms 1

Important Associated Conditions

Antiphospholipid Syndrome

• Antiphospholipid antibodies are present in a subset of patients and increase risk of thrombosis and pregnancy complications 1, 2
• These patients require specific management including consideration of low-dose aspirin for primary prevention 1

Increased Comorbidity Risk

• Osteoporosis prevalence ranges from 4% to 24%, with vertebral fracture prevalence between 7.6% and 37% 1
• Cancer incidence is increased, particularly hematological malignancies, cervical cancer, breast cancer, and lung cancer 1
• Infection risk is elevated due to both disease-related immune dysregulation and immunosuppressive therapy 1

Clinical Pitfalls to Recognize

• The clinical picture is extremely variable between patients and may change over time within the same patient 1
• Disease activity must be distinguished from organ damage, drug toxicity effects, and intercurrent infections 1
• Approximately 12% of patients have some degree of renal activity that may be clinically silent, necessitating regular laboratory monitoring 4
• Patients typically have multiple organ systems involved simultaneously, with more than 50% having 3 or more active organ systems at any given time 4