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
利益声明
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.
鸣谢
Professor Michael Kirschfink would like to gratefully acknowledge Dr D. J. Unsworth, a previous contributor to this topic.
利益声明
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).
同行评议者
Charles Kirkpatrick, MD (UCHSC)
Professor of Medicine
Director
Adult Immunodeficiency Program
University of Colorado Denver
Aurora
CO
利益声明
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.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
参考文献
关键文献
Schröder-Braunstein J, Kirschfink M. Complement deficiencies and dysregulation: Pathophysiological consequences, modern analysis, and clinical management. Mol Immunol. 2019 Oct;114:299-311. 摘要
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. 摘要
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.全文 摘要
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.全文 摘要
参考文献
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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