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Systemic lupus erythematosus

ORPHA536
Synonym(s) Disseminated lupus erythematosus
SLE
Prevalence 6-9 / 10 000
Inheritance Not applicable
Age of onset Adult
ICD-10
  • M32.0
  • M32.1
  • M32.8
  • M32.9
OMIM
UMLS
  • C0024141
MeSH
  • D008180
MedDRA
  • 10042945

Summary

Systemic lupus erythematosus (SLE) is a clinically heterogeneous disease, which is autoimmune in origin and is characterized by the presence of autoantibodies directed against nuclear antigens. It is a multi-system disease, and patients can present in vastly different ways. Prevalence varies with ethnicity, but is estimated to be about 1 per 1000 overall with a female to male ratio of 10:1. The clinical heterogeneity of this disease mirrors its complex etiopathogenesis, which highlights the importance of genetic factors and individual susceptibility to environmental factors. SLE can affect every organ in the body. The most common manifestations include rash, arthritis and fatigue. At the more severe end of the spectrum, SLE can cause nephritis, neurological problems, anemia and thrombocytopenia. Over 90% of patients with SLE have positive anti-nuclear antibodies (ANA). Significant titers are accepted to be of 1:80 or greater. SLE is a relapsing and remitting disease, and treatment aims are threefold: managing acute periods of potentially life-threatening ill health, minimizing the risk of flares during periods of relative stability, and controlling the less life-threatening, but often incapacitating day to day symptoms. Hydroxychloroquine and non-steroidal anti-inflammatory drugs are used for milder disease; corticosteroids and immunosuppressive therapies are generally reserved for major organ involvement; anti-CD20 monoclonal antibody is now used in patients with severe disease who do not respond to conventional treatments. Despite enormous improvements in prognosis since the introduction of corticosteroids and immunosuppressive drugs, SLE continues to have a significant impact on the mortality and morbidity of those affected.

Expert reviewer(s)

  • Dr Jessica MANSON
  • Dr Anisur RAHMAN

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Detailed information

Summary information
Review article
  • EN (2006)
Clinical practice guidelines
  • EN (2012,pdf)
  • FR (2010,pdf)
Article for general public
  • FR (2008,pdf)
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