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Pathogenesis

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

The cause is unknown. It is thought to be multifactorial involving genetic and environmental elements with female sex strongly influencing pathogenesis (1).

Recognised genetic and epidemiological predisposing factors include:

  • hereditary:
    • the concordance for identical twins is 30% compared to 5% for other sibs
  • complement deficiencies:
    • the strongest disease susceptibility genes are those associated with homozygous complement deficiencies (C1q, C1r, C1s, C2 and C4)
  • female sex
    • female hormones are thought to play an important role although the mechanism is unclear (3)
  • associated with HLA-DR2, HLA-DR3 and C4A null complement alleles
  • a particular MHC haplotype that is commonly found in Caucasian patients with SLE is (HLA-A1, HLA-B8, HLA-DR3, C4AQ*O); this is often a marker of a subset of the disease that is associated with anti-Ro antibodies
  • drugs e.g. hydralazine, procainamide or griseofulvin
  • environmental influences
    • Epstein–Barr virus (EBV) infection (4)
    • ultraviolet light (associated with the presence of the anti-Ro antibody) *(4)

Note:

  • *sunlight and ultraviolet light have been thought to be causative factors in development of SLE and of flares but this is not supported by the most recent study (5)

References:

  1. Costenbader KH, Feskanich D, Stampfer MJ, et al. Reproductive and menopausal factors and risk of systemic lupus erythematosus in women. Arthritis Rheum. 2007 Apr;56(4):1251-62.
  2. Teruel M, Alarcón-Riquelme ME. The genetic basis of systemic lupus erythematosus: what are the risk factors and what have we learned. J Autoimmun. 2016 Nov;74:161-75.
  3. Barber M et al. The global epidemiology of SLE: narrowing the knowledge gaps. Rheumatology (Oxford). 2023 Apr; 62(Suppl.1): i4–i9.
  4. Hanlon P, Avenell A, Aucott L, et al. Systematic review and meta-analysis of the sero-epidemiological association between Epstein-Barr virus and systemic lupus erythematosus. Arthritis Res Ther. 2014 Jan 6;16(1):R3.
  5. Tanner TI, Agalliu I, Wahezi DM, et al. Relationship of ultraviolet light exposure and cutaneous and systemic disease activity in youth with childhood-onset systemic lupus: Results from the Childhood Arthritis and Rheumatology Research Alliance Registry. Res Sq [Preprint]. 2024 Jan 3:rs.3.rs-37777

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