Lupus Erythematosus, Systemic (SLE)

BASICS

DESCRIPTION

Multisystem autoimmune inflammatory disorder with variable presentation, disease course, and prognosis
Can manifest in any organ system, especially dermatologic, renal, hematologic, musculoskeletal, cardiovascular
Synonyms: lupus, systemic lupus erythematosus (SLE)

EPIDEMIOLOGY

Incidence

5 to 7 per 100,000 person-years in United States.
Significant variation based on ethnicity: black, 16.0; American Indian/Alaska Native, 7.4; Hispanic, 5.6; Asian/Pacific Islander, 4.6; and white, 3.3 per 100,000 person-years
Strong female predominance compared to male: 9.8 vs 0.8 per 100,000 person-years (~12-fold higher)
Peak incidence: 3rd to 7th decades of life for females, 5th to 7th decades of life for males

Prevalence

Ranges from 20 to 150 cases per 100,000 people in United States.
Strong female predominance: 179 versus 21 cases per 100,000 (~9-fold higher) with highest in black women: 498 cases per 100,000 people

ETIOLOGY AND PATHOPHYSIOLOGY

Genetic, environmental, immunoregulatory, hormonal, and epigenetic factors all play a role.

Genetics

>90 susceptibility loci for SLE in genome-wide association studies (1)[C]

RISK FACTORS

Ethnicity: highest risk in black populations; intermediate risk in Asian, Hispanic, and American Indian/Alaska Native populations
Hormonal: female sex, early menarche/menopause, endometriosis, surgical menopause
Family history of SLE or autoimmune disease
Environmental: cigarette smoking, crystalline silica exposure, exogenous female hormones, certain medications (drug-induced lupus), UV light

COMMONLY ASSOCIATED CONDITIONS

Antiphospholipid syndrome, depression, fibromyalgia, thyroid disease, connective tissue disease syndromes (such as rheumatoid arthritis, Sjögren syndrome, systemic sclerosis)

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