Wegener's granulomatosis and Henoch-Schönlein purpura in a family with hereditary C4 deficiency

K Lhotta; F Kronenberg; M Joannidis; H Feichtinger; P König

doi:10.1007/978-1-4757-9182-2_72

Wegener's granulomatosis and Henoch-Schönlein purpura in a family with hereditary C4 deficiency

Adv Exp Med Biol. 1993:336:415-8. doi: 10.1007/978-1-4757-9182-2_72.

Authors

K Lhotta¹, F Kronenberg, M Joannidis, H Feichtinger, P König

Affiliation

¹ Dept. of Internal Medicine, Innsbruck University Hospital, Austria.

PMID: 8296646
DOI: 10.1007/978-1-4757-9182-2_72

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.

Publication types

Case Reports

MeSH terms

Adolescent
Complement C4 / deficiency*
Complement C4 / metabolism
Granulomatosis with Polyangiitis / etiology*
HLA Antigens / analysis
Haplotypes
Humans
IgA Vasculitis / etiology*
Male
Middle Aged
Phenotype

Substances

Complement C4
HLA Antigens