Summary
Details
- Introduction
- Complement physiology
- Inherited complement deficiencies
- Epidemiology
- Clinical presentation
- Diagnostic tests
- Management
- Guidelines
Contributors
Authors
Michael Kirschfink, DVM, PhD
Professor of Immunology
Consultant Immunologist
German Society of Immunology
International Union of Immunological Societies
International Complement Society
Institute of Immunology
University of Heidelberg
Heidelberg
Germany
Disclosures
MK has sat on Roche and Euro Diagnostica advisory boards; presented a lecture for Novartis; and acted as a consultant for Back Bay Life Science Advisors, Biotest, Ionis Pharmaceuticals, Fresenius Care Germany, and Ablynx. MK is an author of references cited in this topic.
Acknowledgements
Professor Michael Kirschfink would like to gratefully acknowledge Dr D. J. Unsworth, a previous contributor to this topic.
Disclosures
DJU owns shares in Glaxo and Shire, and has had registration fees paid for CPD attendance at international conferences by ALK-Abelló (2015 - EACCI - Allergy) and CSL - Bearing (2016 - ESID - Immunodeficiency).
Peer reviewers
Charles Kirkpatrick, MD (UCHSC)
Professor of Medicine
Director
Adult Immunodeficiency Program
University of Colorado Denver
Aurora
CO
Disclosures
CK is an author of a reference cited in this topic. CK has received speaker fees from Cention, Aventis Behring, and Bristol Myers Squibb. CK has received honorarium for speaking at the Aspen Allergy Conference, for the Colorado Allergy Society. CK ran the research program for a biotechnology company with an approximate budget of $3.5 million.
References
Key articles
Schröder-Braunstein J, Kirschfink M. Complement deficiencies and dysregulation: Pathophysiological consequences, modern analysis, and clinical management. Mol Immunol. 2019 Oct;114:299-311. Abstract
Grumach AS, Kirschfink M. Are complement deficiencies really rare? Overview on prevalence, clinical importance and modern diagnostic approach. Mol Immunol. 2014 Oct;61(2):110-7. Abstract
Maurer M, Magerl M, Betschel S, et al. The international WAO/EAACI guideline for the management of hereditary angioedema-The 2021 revision and update. Allergy. 2022 Jul;77(7):1961-90.Full text Abstract
Brodszki N, Frazer-Abel A, Grumach AS, et al. European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) complement guideline: deficiencies, diagnosis, and management. J Clin Immunol. 2020 May;40(4):576-91.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
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