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HEREDITARY DEFICIENCY OF THE THIRD COMPONENT OF COMPLEMENT IN TWO SISTERS WITH SYSTEMIC LUPUS ERYTHEMATOSUS-LIKE SYMPTOMS

✍ Scribed by Yuji Sano; Hiroaki Nishimukai; Hajime Kitamura; Kazuyoshi Nagaki; Shinya Inai; Yasuteru Hamasaki; Ikuro Maruyama; Akihiro Igata


Publisher
John Wiley and Sons
Year
1981
Tongue
English
Weight
514 KB
Volume
24
Category
Article
ISSN
0004-3591

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✦ Synopsis


Abstract

We observed two sisters with lupus‐like syndrome with homozygous C3 deficiencies. A 19‐year‐old woman and her 15‐year‐old sister developed malar rash, arthralgia, and photosensitivity, but antinuclear antibodies and LE cell preparations were negative. The older sister experienced recurrent bronchitis in her childhood, but the younger sister had no recurrent infections. Serum C3 was not detected immunochemically in either sister, and total complement activity and C3 hemolytic activity were extremely low.


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