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.
KeywordsCrescentic Glomerulonephritis Alternative Complement Pathway Vasculitic Disorder SchOnlein Purpura
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- Fielder, A.H.L., Walport, M. J., Batchelor, J.R., Rynes, R.A., Black, C.M., Dodi, I.A., Hughes, G.R.V., 1983, Family study of the major histocompatibility complex in patients with systemic lupus erythematosus: importance of null alleles of C4A and C4B in determining disease susceptibility, British Medical Journal 286: 425–428PubMedCrossRefGoogle Scholar
- Sim, E., Cross, S.J., 1986, Phenotyping of human complement C4, a class III HLA antigen, Biochem. 239: 763–767Google Scholar