[Hereditary angioneurotic edema: pathophysiology and therapy]

The hereditary angioneurotic edema (HANE), owing to its characteristic clinical symptom, episodic formation of localized edema, has been the subject to be discussed in relation to the problems of the mechanism for the edema formation. On the other hand, when HANE is considered from the point of view on the abnormality in the plasma proteinase systems, it has been thought to be a suitable hereditary defect which enables to investigate into the interactions of the plasma proteinase systems. However, when the clinical course of the disease is correlated with the changes in the plasma proteinase systems, it appears very likely to state that HANE is a very unique model disease, in which edema attacks are elicited by the interactions between plasma proteinase systems and vascular system of the microcirculation, that are triggered by the exogenous stimuli under the influence of the control functions of the endocrine systems.