C1 esterase inhibitor deficiency acquired

This may be a familial (see Hereditary angioneurotic oedema) or, more rarely, an acquired disorder involving the complement system.The acquired form is mostly associated with a B-lymphocyte malignancy, and antibodies have been detected against abnormal immunoglobulins present on the malignant B-cells. Reaction between the two cause C1 activation, which in turn produces a secondary reduction in the concentrations of C1, C2 and C4 and a reduced functional activity of C1 esterase inhibitor (Geha et al 1985).This form must be distinguished from the physical forms of angioedema that occur in response to food, drugs or insect bites, or in association with connective tissue disorders. Recently, a number of patients have developed angioedema in response to treatment with ACE inhibitors, particularly enalapril and captopril (Barna & Frable 1990). Substantial increases in plasma bradykinin have been demonstrated during attacks of hereditary, acquired, and captopril-induced angioneurotic oedema (Nussberger et al 1998).

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