Abstract | PURPOSE: 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:
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Authors | 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 |
Journal | The American journal of medicine
(Am J Med)
Vol. 113
Issue 3
Pg. 194-9
(Aug 15 2002)
ISSN: 0002-9343 [Print] United States |
PMID | 12208377
(Publication Type: Comparative Study, Journal Article)
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Chemical References |
- Antibodies, Anti-Idiotypic
- Complement C1 Inactivator Proteins
- Immunoglobulin A
- Immunoglobulin G
- Immunoglobulin M
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Topics |
- Age Distribution
- Aged
- Angioedema
(diagnosis, epidemiology, immunology)
- Antibodies, Anti-Idiotypic
(analysis, immunology)
- Blotting, Western
- Cohort Studies
- Comorbidity
- Complement C1 Inactivator Proteins
(analysis, immunology)
- Enzyme-Linked Immunosorbent Assay
- Female
- Follow-Up Studies
- Humans
- Immunoglobulin A
(analysis)
- Immunoglobulin G
(analysis)
- Immunoglobulin M
(analysis)
- Lymphoproliferative Disorders
(epidemiology, immunology)
- Male
- Middle Aged
- Paraproteinemias
(diagnosis, epidemiology)
- Prevalence
- Risk Assessment
- Sex Distribution
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