International Archives of Allergy and Immunology
Original Paper
Acquired Angioedema - Occurrence, Clinical Features and Associated Disorders in a Danish Nationwide Patient CohortBygum A.a · Vestergaard H.baDepartment of Dermatology and Allergy Centre, and bDepartment of Haematology, Odense University Hospital, Odense, Denmark
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Article / Publication Details
Received: January 31, 2013
Accepted: April 12, 2013
Published online: July 31, 2013
Issue release date: September 2013
Number of Print Pages: 7
Number of Figures: 0
Number of Tables: 2
ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)
For additional information: https://www.karger.com/IAA
Abstract
Background: The prevalence of acquired angioedema (AAE) is hitherto unknown and, to date, less than 200 patients have been reported worldwide. AAE is associated with lymphoproliferative conditions and autoantibodies against C1 inhibitor (C1INH). Rituximab (RTX) is increasingly used in the treatment of AAE patients. Methods: A nationwide study of AAE patients was performed in Denmark. Clinical features, associated disorders, treatments and outcomes were registered. Results: Eight AAE patients were identified. The diagnostic delay was on average 1 year and 8 months. Patients were treated with C1INH concentrate or icatibant on demand. Six patients were diagnosed with a clonal B-cell disorder during follow-up, on average 2.5 years after the first swelling. Two patients had monoclonal B-cell lymphocytosis (MBL). Two patients received RTX. Conclusions: AAE is a rare condition occurring in less than 10% of patients with C1INH deficiency in Denmark. AAE is highly associated with haematologic disorders, and we recommend yearly follow-up visits with clinical examination and blood tests including flow cytometry to diagnose B-cell conditions at an early stage. We report 2 patients with AAE and associated MBL, which is a benign expansion of clonal B lymphocytes. MBL can be the precursor of chronic lymphocytic leukaemia or is associated with non-Hodgkin's lymphoma. If angioedema is poorly controlled with standard treatment regimens, we suggest treatment of the associated haematologic disorder. Based on a review of the literature and our own data, we recommend therapy with RTX, especially in patients with anti-C1INH autoantibodies.
© 2013 S. Karger AG, Basel
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Article / Publication Details
Received: January 31, 2013
Accepted: April 12, 2013
Published online: July 31, 2013
Issue release date: September 2013
Number of Print Pages: 7
Number of Figures: 0
Number of Tables: 2
ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)
For additional information: https://www.karger.com/IAA
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