Complement deficiencies
Authors:
Marta Sobotková
Authors‘ workplace:
Ústav imunologie 2. LF UK a FN v Motole
Published in:
Vnitř Lék 2020; 66(6): 346-352
Category:
Main Topic
Overview
Complement system plays a crucial role in innate imunity. Complement deficiencies are often associated with severe infections, usually meningoccocal, pneumococcal or caused by Haemophilus influenzae, or with autoimmune diseases, especially systemic lupus erythematodes. Inherited complement deficiencies are very rare although their prevalence in population may be underestimated due to lower availability of adequate laboratory testing. Acquired complement deficiencies accompany other underlying diseases and often are caused by increased consumption and only partial.
Keywords:
infection – Complement – complement deficiency – autoimmune disease
Sources
1. Krejsek J, Andrýs C, Krčmová I. Imunologie člověka, Garamon: Hradec Králové, 2016. 495 stran. ISBN 978‑80‑86472‑74‑4
2. Schröder ‑Braunstein J, Kirschfink M, et al. Complement deficiencies and dysregulation: Pathophysiological consequences, modern analysis, and clinical management. Mol Immunol. 2019; 114: 299–311.
3. Hořejší V, Bartůňková J, Brdička T, et al. Základy imunologie. 6., aktualizované vydání, Triton: Praha 2017: 51–57. ISBN 979‑80‑7553‑250‑3
4. Mayilyan KR. Complement genetics, deficiencies, and disease associations. Protein Cell. 2012; 3(7):487–496.
5. 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; 40(4): 576–591.
6. Ricklin D, Lambris JD. Complement in immune and inflammatory disorders: pathophysiological mechanisms. J Immunol. 2013; 190(8): 3831–3838.
7. Liszewski MK, Atkinson JP. Inherited disorders of the compleemnt system. Up ‑to‑date [Cit. 2020‑07‑08]. dostupné z WWW: https://www.uptodate.com/contents/ inherited ‑disorders ‑of ‑the ‑complement ‑system/print?search=complement%20defi‑ ciencies & source=search_result & selectedTitle=1~107 & usage_type=default & dis‑ play_rank=1
8. Liszewski MK, Atkinson JP. Acquired deficiencies of the complement systém. Up‑to‑date [Cit. 2020‑07‑08]. dostupné z WWW: https://www.uptodate.com/con‑ tents/acquired ‑deficiencies ‑of ‑the ‑complement ‑system/print?search=complement%20deficiencies & source=search_result & selectedTitle=2~107 & usage_type=default & display_rank=2
9. Tangye SG, Al‑Herz W, Bousfiha A, et al. Human Inborn Errors of Immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2020; 40(1): 24–64.
Labels
Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
2020 Issue 6
Most read in this issue
- Gastrointestinal tract involvement in mixed connective tissue disease (Sharp syndrome)
- Myocardial infarction or broken heart syndrome?
- Food allergy and intolerance
- Complement deficiencies