Wegener's granulomatosis and Henoch-Schönlein purpura in a family with hereditary C4 deficiency.
Described here is a family in which the father with partial C4 deficiency had Wegener's granulomatosis and a son with complete C4 deficiency presented with Henoch-Schönlein Purpura. The possible role of C4 deficiency in the pathogenesis of both vasculitic disorders is discussed.
Lhotta
K
K
Dept. of Internal Medicine, Innsbruck University Hospital, Austria.
Kronenberg
F
F
Joannidis
M
M
Feichtinger
H
H
König
P
P
D000293:Adolescent; D003181:Complement C4 / Q000172:deficiency* / Q000378:metabolism; D014890:Granulomatosis with Polyangiitis / Q000209:etiology*; D006680:HLA Antigens / Q000032:analysis; D006239:Haplotypes; D006801:Humans; D011695:IgA Vasculitis / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D010641:Phenotype
D003181:Complement C4; D006680:HLA Antigens