Baseline level of functional C1-inhibitor correlates with disease severity scores in hereditary angioedema |
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| Authors: | Zsuzsanna Kelemen Dumitru Moldovan Enikő Mihály Beáta Visy Gábor Széplaki Dorottya Csuka George Füst Henriette Farkas Lilian Varga |
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| Affiliation: | 1. 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary;2. Internal Medicine Clinic, University of Medicine and Pharmacy Tîrgu Mure?, Allergology and Clinical Immunology Department, Mures County Hospital, Tîrgu Mure?, Romania;3. Romanian Haereditary Angioedema Network, Tirgu Mure?, Romania;4. Heim Pál Children Hospital, Budapest, Hungary;1. Department of Allergy, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China;2. Department of Allergy, Tianjin Medical University General Hospital, Tianjin, China;3. Department of Medicine and Pediatrics, Penn State University, Hershey, Pennsylvania;1. Allergy Department, Hospital La Paz Institute for Health Research (IdiPaz), Paseo de la Castellana 261, Madrid 28046, Spain;2. CIBERER (U754), Allergy Department, Hospital La Paz Institute for Health Research (IdiPaz), Paseo de la Castellana 261, Madrid 28046, Spain;3. Allergy Department, Hospital Universitario Severo Ochoa, Avenida de Orellana s/n, Leganés, Madrid 28911, Spain;1. University of California–San Diego, La Jolla, California;2. Massachusetts General Hospital, Boston, Massachusetts;3. Icahn School of Medicine at Mount Sinai, New York, New York;4. University of North Carolina–Charlotte, Charlotte, North Carolina;6. Winthrop Rheumatology Allergy & Immunology, Mineola, New York;5. CSL Behring, King of Prussia, Pennsylvania;1. Division of Allergy, Immunology, and Rheumatology, Department of Medicine, University of California, San Diego, La Jolla, Calif;2. Allergy and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, Mass;3. Marycliff Allergy Specialists, Spokane, Wash |
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| Abstract: | The diagnosis of hereditary angioedema (HAE) is based on complement tests. We studied for the first time the possible association between complement parameters measured at the time of diagnosis and disease severity in 115 patients with HAE.Serum levels of functional C1-inhibitor (C1-INHf), antigenic C1-inhibitor (C1-INHa), C4 and hemolytic activity of the classical pathway (CH50) were determined at the time of diagnosis. We found a significant correlation between severity scores and baseline C1-INHf levels, as determined by ELISA assay (p = 0.0003). On the other hand, there was no correlation between severity scores and other complement parameters (C1-INHa, C4, and CH50).We consider the correlation between severity scores and baseline C1-INHf levels an important pathophysiological observation. Our findings underlie the potential significance of monitoring functional C1-INH levels in relation to clinical disease course. |
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