Lupus Erythematosus, Systemic (SLE)

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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; SLE

Epidemiology

Incidence

  • 5 to 12 per 100,000 person-years in United States
  • Significant variation based on ethnicity: Black 16, American Indian/Alaska Native 7.4, Hispanic 5.6, Asian/Pacific Islander 4.6, White 3.3 per 100,000 person-years
  • Strong female predominance: 9.8 versus 0.8 per 100,000 person-years (~12× higher)
  • Peak incidence: 3rd to 7th decades of life for females, 5th to 7th decades for males

Prevalence

  • Ranges from 20 to 150 cases per 100,000 in United States
  • Strong female predominance: 179 versus 21 cases per 100,000 (~9× higher) with highest in Black women: 498 per 100,000

Etiology and Pathophysiology

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

Genetics

Over 100 gene loci susceptible 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, viral infections, vitamin D deficiency, alfalfa sprouts, foods containing canavanine

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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