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Acquired C1 inhibitor deficiency: Management and prognosis

Marco Cicardi, MD
Section Editor
Sarbjit Saini, MD
Deputy Editor
Anna M Feldweg, MD


Acquired angioedema due to deficiency of C1 esterase inhibitor, also called acquired angioedema and abbreviated C1INH-AAE, is a rare syndrome of recurrent episodes of angioedema, without urticaria, which is associated with B cell lymphoproliferative disorders in some patients [1]. Angioedema typically affects the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. The swelling is self-limited, although laryngeal involvement may cause fatal asphyxiation. Clinically, this disorder is very similar to hereditary angioedema, although acquired angioedema develops in older patients and is frequently associated with underlying disease, whereas the hereditary disorder presents in younger patients who are otherwise healthy [2].

This topic review will discuss the management and prognosis of C1INH-AAE. The clinical manifestations, epidemiology, pathogenesis, and diagnosis of this disorder are reviewed elsewhere. (See "Acquired C1 inhibitor deficiency: Clinical manifestations, epidemiology, pathogenesis, and diagnosis".)

Hereditary angioedema, which is caused by mutations in the gene for C1INH, is discussed separately. (See "Hereditary angioedema: Pathogenesis and diagnosis" and "Hereditary angioedema: Epidemiology, clinical manifestations, exacerbating factors, and prognosis" and "Hereditary angioedema: Treatment of acute attacks" and "Hereditary angioedema: General care and long-term prophylaxis".)


The management of a patient with acquired angioedema involves several components [3]:

Educating the patient about the potential triggers and early recognition of angioedema attacks and ensuring that the patient understands that there is a real risk of fatal asphyxiation with attacks involving the upper airway. (See 'Patient education about laryngeal edema' below and 'Avoidance of exacerbating factors' below.)

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Literature review current through: Oct 2017. | This topic last updated: Apr 11, 2016.
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