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Naturally occurring variation in gene copy number is increasingly recognized as a heritable source of susceptibility to genetically complex diseases. Here we report strong association between FCGR3B copy number and risk of systemic lupus erythematosus (P = 2.7 x 10(-8)), microscopic polyangiitis (P = 2.9 x 10(-4)) and Wegener's granulomatosis in two independent cohorts from the UK (P = 3 x 10(-3)) and France (P = 1.1 x 10(-4)). We did not observe this association in the organ-specific Graves' disease or Addison's disease. Our findings suggest that low FCGR3B copy number, and in particular complete FCGR3B deficiency, has a key role in the development of systemic autoimmunity.

Original publication




Journal article


Nat Genet

Publication Date





721 - 723


Antigens, CD, Autoimmune Diseases, Autoimmunity, Disease Susceptibility, France, GPI-Linked Proteins, Gene Dosage, Genetic Predisposition to Disease, Genotype, Granulomatosis with Polyangiitis, Humans, Lupus Erythematosus, Systemic, Receptors, IgG, United Kingdom