Systemic Lupus Erythematosus (SLE)

  • multifactorial autoimmune disease that mostly affects young women

SLE is part of a spectrum

Lupus Spectrum
  • SLE is one component of the lupus spectrum
  • Adult or paediatric patients with nephritis also have SLE, but other lupus syndromes incompletely overlap with SLE itself.
  • Incomplete lupus and cutaneous LE may be subsets that include individuals who will eventually develop SLE.
    • Cutaneous lesions occur in up to 85% of patients with SLE and are the first sign in up to 28%
  • MCTD has distinct elements outside of the usual lupus spectrum, such as erosive arthritis.
  • Anti-phospholipid syndrome may exist as a separate entity or may be part of SLE

Clinical

  • Triggers for flares include the following:
    • infections, ultraviolet irradiation,
    • medications such as quinidine, hydralazine and procainamide
    • pregnancy
  • Premature atherosclerosis is an important cause of morbidity and mortality in SLE
  • Metabolic syndrome - a clustering of cardiovascular risk factors associated with insulin resistance and an increased risk of future type 2 diabetes mellitus and cardiovascular disease
  • Adult SLE patients are at risk of glucocorticoid-induced osteoporosis
  • Lupus enteritis, including GI vasculitis, is common with or without infarction
    • prevalence of intestinal vasculitis variously reported up to 53%

Management

  • antimalarials - in particular hydroxychloroquine
  • pulse therapy with steroids occasionally - often used but evidence is lacking for high dose therapy
  • immuno-suppressants - azathioprine does have steroid sparing effects, cyclophosphamide, Mycophenolate mofetil
  • biologics - Rituximab, Belimumab, Silfalimumab

References include
abdo pain in lupus
review of diagnosis and Mx 2016