Acquired angioneurotic edema: biological and clinical features in nine patients

OBJECTIVE: Angioneurotic edema (AE) is a rare but severe disease, Hereditar y AE is the more well-known form. The acquired form is I exceptional: the s ymptoms are the same but i there are some biologic and treatment difference s. We investigated the clinical and biochemical features in nine patients w ith acquired angioneurotic edema (AAE). PATIENTS AND METHODS: Four of the patients with type I AAE presented an acc elerated metabolism of C1lnh, associated with a hematology disease. Their C 4, C1q and C1lnh plasma levels were low. Four patients had type II AAE asso ciated with an autoantibody to C1lnh. Their C1lnh plasma levels were normal or low but the functional levels were low in all four. One patient had AAE induced by oral contraceptives The C1lnh plasma level was normal but the f unctional level was very low; there were no autoantibodies. Symptoms resolv ed when oral contraceptives were withdrawn and the C1lnh level returned to normal. DISCUSSION: Treatment of AAE is a difficult matter. For type I AAE, it cons ists in treating the associated disease. For type II AAE, the treatment goa l is to lower the autoantibody level. Management of these diseases requires close collaboration between clinicians and biologists.