title: Wegener's granulomatosis and Henoch-Schönlein purpura in a family with hereditary C4 deficiency.
abstract: 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.
authors.affiliation: Dept. of Internal Medicine, Innsbruck University Hospital, Austria.
authors.forename: K
authors.initials: K
authors.lastname: Lhotta
authors.forename: F
authors.initials: F
authors.lastname: Kronenberg
authors.forename: M
authors.initials: M
authors.lastname: Joannidis
authors.forename: H
authors.initials: H
authors.lastname: Feichtinger
authors.forename: P
authors.initials: P
authors.lastname: König
mesh_terms: 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
chemical_list: D003181:Complement C4; D006680:HLA Antigens