Acquired C1 Esterase Inhibitor Deficiency and Angioedema

Acquired C1 inhibitor (C1-INH) deficiency type II. Replacement therapy with C1-INH and analysis of patients' C1-INH and anti-C1-INH autoantibodies.

“…In humans a functional deficiency of Cl-INH is associated with complement activation and recurrent angioedema (2,3). The syndrome ofhereditary angioedema (HAE) is the most common form of this disease and is related either to a decreased synthesis of apparently normal Cl-INH protein (HAE type I) (4)(5)(6) or to the presence of a'dysfunctional protein (HAE type EI) (7)(8)(9) (10)(11)(12)(13). Both HAE and AAE are characterized by low levels or absence of C2, C4, and functionally active CI-INH (6,10,14).…”

“…The syndrome ofhereditary angioedema (HAE) is the most common form of this disease and is related either to a decreased synthesis of apparently normal Cl-INH protein (HAE type I) (4)(5)(6) or to the presence of a'dysfunctional protein (HAE type EI) (7)(8)(9) (10)(11)(12)(13). Both HAE and AAE are characterized by low levels or absence of C2, C4, and functionally active CI-INH (6,10,14). In contrast to the hereditary form, patients with AAE' show a marked decrease of Cl levels, a normal or slightly increased CI-INH synthesis, and an onset of symptoms in middle age (10,13).…”

Demonstration of modified inactive first component of complement (C1) inhibitor in the plasmas of C1 inhibitor-deficient patients.

“…Three distinct syndromes of Cl inhibitor deficiency occur in humans and are associated with complement activation and recurrent angioedema: hereditary angioedema (HAE)' (1) and variant HAE (2) are inherited in an autosomal dominant manner; and acquired Cl inhibitor deficiency (3)(4)(5) usually occurs in conjunction with a lymphoproliferative disease or paraproteinemia. The pathogenesis of edema formation in the C inhibitordeficiency syndromes is not completely understood.…”

Hereditary angioneurotic oedema, tracheal intubation and airway obstruction

“…5 A patient undergoing eholeeystectomy has also been described. 6 The anaesthetic technique is not mentioned but it is likely to have included tracheal intubation.…”