Document Detail

Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2.
MedLine Citation:
PMID:  12208377     Owner:  NLM     Status:  MEDLINE    
PURPOSE: Acquired angioedema type 1 is characterized by a C1 inhibitor deficiency in patients with lymphoproliferative disorders, whereas acquired angioedema type 2 is characterized by anti-C1 inhibitor antibodies, and has not been thought to be associated with lymphoproliferative disease. We studied the clinical features, complement profiles, and associated diseases in 19 new patients with diagnosed acquired angioedema type 2. SUBJECTS AND METHODS: Plasma concentrations and functional activity of complement components were measured by conventional techniques. Functional C1 inhibitor activity was assessed by a chromogenic assay. Autoantibodies to C1 inhibitor were detected using an enzyme-linked immunosorbent assay. RESULTS: The 11 men and 8 women (median age, 60 years) presented with recurrent attacks of angioedema. All patients had detectable anti-C1 inhibitor antibodies in serum. A monoclonal gammopathy was detected in 12 patients (63%) at the time of diagnosis, 11 of whom had an immunoglobulin peak of the same heavy- and light-chain isotypes as the acquired anti-C1 inhibitor antibody. Three of these 12 patients developed a malignant lymphoproliferative disease. CONCLUSION: As with type 1 disease, a large proportion of patients with acquired angioedema type 2 have a lymphoproliferative disorder.
Véronique Frémeaux-Bacchi; Marie-Thérèse Guinnepain; Patrice Cacoub; Marie-Agnès Dragon-Durey; Luc Mouthon; Jacques Blouin; Patrick Cherin; Jérome Laurent; Jean-Charles Piette; Wolf-H Fridman; Laurence Weiss; Michel O Kazatchkine
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  The American journal of medicine     Volume:  113     ISSN:  0002-9343     ISO Abbreviation:  Am. J. Med.     Publication Date:  2002 Aug 
Date Detail:
Created Date:  2002-09-04     Completed Date:  2002-09-17     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0267200     Medline TA:  Am J Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  194-9     Citation Subset:  AIM; IM    
Département d'Immunologie, Hôpital Européen Georges Pompidou, Paris, France.
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MeSH Terms
Age Distribution
Angioedema / diagnosis,  epidemiology*,  immunology*
Antibodies, Anti-Idiotypic / analysis,  immunology*
Blotting, Western
Cohort Studies
Complement C1 Inactivator Proteins / analysis,  immunology*
Enzyme-Linked Immunosorbent Assay
Follow-Up Studies
Immunoglobulin A / analysis
Immunoglobulin G / analysis
Immunoglobulin M / analysis
Lymphoproliferative Disorders / epidemiology,  immunology
Middle Aged
Paraproteinemias / diagnosis*,  epidemiology*
Risk Assessment
Sex Distribution
Reg. No./Substance:
0/Antibodies, Anti-Idiotypic; 0/Complement C1 Inactivator Proteins; 0/Immunoglobulin A; 0/Immunoglobulin G; 0/Immunoglobulin M
Comment In:
Am J Med. 2002 Aug 15;113(3):249-51   [PMID:  12208389 ]

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