Abstract
Angioedema may be due to hereditary forms of Cl-Inh deficiency, but recently an autoimmune form of angioedema has been described in which the mechanism is novel. While the peripheral blood monocytes of patients with autoimmune angioedema produce a normal, functionally active, 105 KD Cl-Inh in normal quantities, the Cl-Inh isolated from the patient's plasma exists in a dysfunctional lower molecular weight (96 KD) performance. Rather than bind and biologically inactivate the enzyme, a relatively common phenomenon in autoimmune disease, the autoimmune angioedema cleave the Cl-Inh molecule. The following sequence of events is proposed: structural and functionally normal Cl-Inh is synthesised and secreted, this secreted inhibitor is complexed by autoantibody and following enzyme interaction, denatured 96 KD Cl-Inh is proposed. This process depletes the pool of normal, functional Cl-Inh to critical levels and predisposes patients to episodes of oedema.
References
Jul 1, 1979·Medicine·J A GelfandM M Frank
May 1, 1976·Annals of Internal Medicine·M M FrankJ P Atkinson
Jun 1, 1988·The Journal of Allergy and Clinical Immunology·A MalbranL F Fries
Jun 1, 1989·The Journal of Clinical Investigation·M AraiL W Hoyer
Feb 1, 1989·The Journal of Clinical Investigation·J JacksonC Feighery
Jun 1, 1989·The Journal of Clinical Investigation·J AlsenzM Loos
Jan 14, 1988·The New England Journal of Medicine·J Jackson, C Feighery
Jan 14, 1988·The New England Journal of Medicine·K P Klugman, H Koornhof
May 28, 1987·The New England Journal of Medicine·J AlsenzM Loos
Oct 23, 1986·Nature·J JacksonC Feighery
Aug 1, 1986·The Journal of Clinical Investigation·B L Zuraw, J G Curd
Feb 28, 1985·The New England Journal of Medicine·R S GehaF S Rosen
Jun 1, 1985·The Journal of Allergy and Clinical Immunology·A L ShefferD T Fearon
Jan 1, 1983·Annual Review of Biochemistry·J Travis, G S Salvesen
Jan 1, 1981·Methods in Enzymology·R B Sim, A Reboul
Jul 1, 1963·The American Journal of Medicine·V H DONALDSON, R R EVANS
Jul 1, 1962·The Journal of Allergy·N S LANDERMANH E RATCLIFFE
Jul 1, 1962·The Journal of Allergy·N S LANDERMAN