A case of C7 deficiency associated with Sjoegren's syndrome, Raynaud's phenomenon and anti-centromere antibody.

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We reported a case of C7 deficiency associated with Sjögren's syndrome, Raynaud's phenomenon and anti-centromere antibody. A 54 years old woman complained of Raynaud's phenomenon, dry mouth and dry eye in 1986. She was pointed out to have keratocojunctivitis sicca, leukocytopenia, anti-centromere antibody, elevated anti DNA antibody and decreased CH 50 activity. Anti-centromere antibody and decreased CH 50 activity was persistent until the present time. Further examination identified the presence of C7 deficiency. In the previously reported cases of C7 deficiency, only 2 SLE, 1 rheumatoid arthritis, 1 ankylosing spondylosis and 1 CREST syndrome cases were noticed. The relationship between Sjögren's syndrome, Raynaud's phenomenon, anti-centromere antibody and C7 deficiency remains unclear in this case.
日本臨床免疫学会の論文