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Complement factor 2 deficiency: a clinical and serological family study.
  1. D D'Cruz,
  2. J Taylor,
  3. T Ahmed,
  4. R Asherson,
  5. M Khamashta,
  6. G R Hughes
  1. Lupus Arthritis Research Unit, Rayne Institute, St Thomas's Hospital, London, United Kingdom.


    Inherited complement deficiencies are associated with a variety of connective tissue diseases. A family with inherited deficiency of complement factor 2 (C2) is described in which two family members with homozygous C2 deficiency developed cutaneous vasculitis and sicca syndrome. The other family members had heterozygous C2 deficiency and each member had the HLA-A25, B18, DR2 (w15) haplotype. The mother had seropositive rheumatoid arthritis. Further studies showed the presence of cryoglobulins, antibodies against endothelial cells, and anticardiolipin antibodies.

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