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Reduced circulating complement concentration

MedGen UID:
82898
•Concept ID:
C0272242
•
Disease or Syndrome
Synonym: Complement deficiency
SNOMED CT: Defects in the complement system (24743004); Complement deficiency disease (24743004)
 
HPO: HP:0004431
Monarch Initiative: MONDO:0003832
Orphanet: ORPHA459345

Definition

An immunodeficiency defined by the absent or suboptimal functioning of one of the complement system proteins. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVReduced circulating complement concentration

Conditions with this feature

Complement component C1r/C1s deficiency
MedGen UID:
461624
•Concept ID:
C3150274
•
Disease or Syndrome
Lack of production of either functional C1r or C1s protein, due to a genetic defect. Approximately 60% of patients with a C1r/C1s deficiency will develop a severe systemic lupus erythematosus at an early age. Patients also present with frequent sinopulmonary infections often with Streptococcus pneumoniae.
See: Condition Record
Immunodeficiency due to MASP-2 deficiency
MedGen UID:
462435
•Concept ID:
C3151085
•
Disease or Syndrome
MASP2 deficiency, classically defined as MASP2 protein level of less than 100 ng/ml, occurs in about 4% of Caucasians and up to 18% of some African populations. Some MASP2-deficient individuals have increased risk of infection or autoimmune disease, but most are asymptomatic. MASP2 plays a role in activation of the lectin pathway of the complement system; deficiency may thus lead to defects in the complement system (summary by Thiel et al., 2007 and Sokolowska et al., 2015). For a discussion of genetic heterogeneity of lectin complement activation pathway defects, see LCAPD1 (614372).
See: Condition Record
Complement component C1r/C1s deficiency
Immunodeficiency due to MASP-2 deficiency

Professional guidelines

PubMed

Extracorporeal therapy in the treatment of sepsis: In vitro assessment of the effect of an absorbent cartridge on the circulating bacterial concentration and its interaction with the antibiotic therapy.
de Cal M, Lorenzin A, Risino B, Zanetti F, Fanton G, Fallico L, Rassu M, De Rosa S, Ronco C, Zanella M
Int J Artif Organs 2023 Jun;46(6):344-350. Epub 2023 Apr 17 doi: 10.1177/03913988231168155. PMID: 37066442
Bempedoic acid in the management of lipid disorders and cardiovascular risk. 2023 position paper of the International Lipid Expert Panel (ILEP).
Banach M, Penson PE, Farnier M, Fras Z, Latkovskis G, Laufs U, Paneni F, Parini P, Pirro M, Reiner Ž, Vrablik M, Escobar C
Prog Cardiovasc Dis 2023 Jul-Aug;79:2-11. Epub 2023 Mar 7 doi: 10.1016/j.pcad.2023.03.001. PMID: 36889490
Inflammation and atherosclerosis: role of C-reactive protein in risk assessment.
Libby P, Ridker PM
Am J Med 2004 Mar 22;116 Suppl 6A:9S-16S. doi: 10.1016/j.amjmed.2004.02.006. PMID: 15050187

Recent clinical studies

Etiology

Circulating mortalin in blood and activation of the alternative complement pathway as risk indicators in COVID-19 infection.
Avraham M, Sinkovits G, Hurler L, Prohászka Z, Fishelson Z
Front Immunol 2024;15:1337215. Epub 2024 Apr 23 doi: 10.3389/fimmu.2024.1337215. PMID: 38715618Free PMC Article
Bempedoic acid in the management of lipid disorders and cardiovascular risk. 2023 position paper of the International Lipid Expert Panel (ILEP).
Banach M, Penson PE, Farnier M, Fras Z, Latkovskis G, Laufs U, Paneni F, Parini P, Pirro M, Reiner Ž, Vrablik M, Escobar C
Prog Cardiovasc Dis 2023 Jul-Aug;79:2-11. Epub 2023 Mar 7 doi: 10.1016/j.pcad.2023.03.001. PMID: 36889490
The effect of soy protein containing soy isoflavones on serum concentration of cell adhesion molecules: A systematic review and meta-analysis of randomized controlled trials.
Hariri M, Baradaran HR, Gholami A
Complement Ther Med 2021 Sep;61:102764. Epub 2021 Jul 29 doi: 10.1016/j.ctim.2021.102764. PMID: 34333131
Tracing surface and airborne SARS-CoV-2 RNA inside public buses and subway trains.
Moreno T, Pintó RM, Bosch A, Moreno N, Alastuey A, Minguillón MC, Anfruns-Estrada E, Guix S, Fuentes C, Buonanno G, Stabile L, Morawska L, Querol X
Environ Int 2021 Feb;147:106326. Epub 2020 Dec 9 doi: 10.1016/j.envint.2020.106326. PMID: 33340987Free PMC Article
Complement factors and circulating immune complexes in children with urinary schistosomiasis and asymptomatic malaria.
Arinola OG
Afr J Med Med Sci 2005 Mar;34(1):9-13. PMID: 15971548

Diagnosis

Bempedoic acid in the management of lipid disorders and cardiovascular risk. 2023 position paper of the International Lipid Expert Panel (ILEP).
Banach M, Penson PE, Farnier M, Fras Z, Latkovskis G, Laufs U, Paneni F, Parini P, Pirro M, Reiner Ž, Vrablik M, Escobar C
Prog Cardiovasc Dis 2023 Jul-Aug;79:2-11. Epub 2023 Mar 7 doi: 10.1016/j.pcad.2023.03.001. PMID: 36889490
Association between Circulating Antioxidants and Longevity: Insight from Mendelian Randomization Study.
Yu Z, Zhang F, Xu C, Wang Y
Biomed Res Int 2022;2022:4012603. Epub 2022 Jan 29 doi: 10.1155/2022/4012603. PMID: 35132376Free PMC Article
Vitamin D Status and Cancer Incidence, Survival, and Mortality.
Kim H, Giovannucci E
Adv Exp Med Biol 2020;1268:39-52. doi: 10.1007/978-3-030-46227-7_3. PMID: 32918213
Pro-inflammatory cytokines and adipose tissue.
Coppack SW
Proc Nutr Soc 2001 Aug;60(3):349-56. doi: 10.1079/pns2001110. PMID: 11681809
On insulin secretion.
Täljedal IB
Diabetologia 1981 Jul;21(1):1-17. doi: 10.1007/BF03216216. PMID: 7024025

Therapy

Bempedoic acid in the management of lipid disorders and cardiovascular risk. 2023 position paper of the International Lipid Expert Panel (ILEP).
Banach M, Penson PE, Farnier M, Fras Z, Latkovskis G, Laufs U, Paneni F, Parini P, Pirro M, Reiner Ž, Vrablik M, Escobar C
Prog Cardiovasc Dis 2023 Jul-Aug;79:2-11. Epub 2023 Mar 7 doi: 10.1016/j.pcad.2023.03.001. PMID: 36889490
Pharmacokinetic considerations to optimize clinical outcomes for COVID-19 drugs.
Nwabufo CK, Bendayan R
Trends Pharmacol Sci 2022 Dec;43(12):1041-1054. Epub 2022 Sep 26 doi: 10.1016/j.tips.2022.09.005. PMID: 36374805Free PMC Article
Association between Circulating Antioxidants and Longevity: Insight from Mendelian Randomization Study.
Yu Z, Zhang F, Xu C, Wang Y
Biomed Res Int 2022;2022:4012603. Epub 2022 Jan 29 doi: 10.1155/2022/4012603. PMID: 35132376Free PMC Article
Imlifidase-generated Single-cleaved IgG: Implications for Transplantation.
Bockermann R, Järnum S, Runström A, Lorant T, Winstedt L, Palmqvist N, Kjellman C
Transplantation 2022 Jul 1;106(7):1485-1496. Epub 2021 Dec 27 doi: 10.1097/TP.0000000000004031. PMID: 34966107Free PMC Article
Vitamin D Status and Cancer Incidence, Survival, and Mortality.
Kim H, Giovannucci E
Adv Exp Med Biol 2020;1268:39-52. doi: 10.1007/978-3-030-46227-7_3. PMID: 32918213

Prognosis

Vitamin D Status and Cancer Incidence, Survival, and Mortality.
Kim H, Giovannucci E
Adv Exp Med Biol 2020;1268:39-52. doi: 10.1007/978-3-030-46227-7_3. PMID: 32918213
Role of interleukins in heart failure with reduced ejection fraction.
Segiet OA, Piecuch A, Mielanczyk L, Michalski M, Nowalany-Kozielska E
Anatol J Cardiol 2019 Nov;22(6):287-299. doi: 10.14744/AnatolJCardiol.2019.32748. PMID: 31789611Free PMC Article
Complement activation, inflammation and relative ADAMTS13 deficiency in secondary thrombotic microangiopathies.
Farkas P, Csuka D, Mikes B, Sinkovits G, Réti M, Németh E, Rácz K, Madách K, Gergely M, Demeter J, Prohászka Z
Immunobiology 2017 Feb;222(2):119-127. Epub 2016 Oct 18 doi: 10.1016/j.imbio.2016.10.014. PMID: 27771173
Complement component C1q as potential diagnostic but not predictive marker of preeclampsia.
Agostinis C, Stampalija T, Tannetta D, Loganes C, Vecchi Brumatti L, De Seta F, Celeghini C, Radillo O, Sargent I, Tedesco F, Bulla R
Am J Reprod Immunol 2016 Dec;76(6):475-481. Epub 2016 Sep 26 doi: 10.1111/aji.12586. PMID: 27666323
Role of complement in immune or idiopathic thrombocytopenic purpura.
Hed J
Acta Paediatr Suppl 1998 Jun;424:37-40. doi: 10.1111/j.1651-2227.1998.tb01231.x. PMID: 9736216

Clinical prediction guides

Circulating mortalin in blood and activation of the alternative complement pathway as risk indicators in COVID-19 infection.
Avraham M, Sinkovits G, Hurler L, Prohászka Z, Fishelson Z
Front Immunol 2024;15:1337215. Epub 2024 Apr 23 doi: 10.3389/fimmu.2024.1337215. PMID: 38715618Free PMC Article
Association between Circulating Antioxidants and Longevity: Insight from Mendelian Randomization Study.
Yu Z, Zhang F, Xu C, Wang Y
Biomed Res Int 2022;2022:4012603. Epub 2022 Jan 29 doi: 10.1155/2022/4012603. PMID: 35132376Free PMC Article
Vitamin D Status and Cancer Incidence, Survival, and Mortality.
Kim H, Giovannucci E
Adv Exp Med Biol 2020;1268:39-52. doi: 10.1007/978-3-030-46227-7_3. PMID: 32918213
Role of interleukins in heart failure with reduced ejection fraction.
Segiet OA, Piecuch A, Mielanczyk L, Michalski M, Nowalany-Kozielska E
Anatol J Cardiol 2019 Nov;22(6):287-299. doi: 10.14744/AnatolJCardiol.2019.32748. PMID: 31789611Free PMC Article
Complement component C1q as potential diagnostic but not predictive marker of preeclampsia.
Agostinis C, Stampalija T, Tannetta D, Loganes C, Vecchi Brumatti L, De Seta F, Celeghini C, Radillo O, Sargent I, Tedesco F, Bulla R
Am J Reprod Immunol 2016 Dec;76(6):475-481. Epub 2016 Sep 26 doi: 10.1111/aji.12586. PMID: 27666323

Recent systematic reviews

The effect of soy protein containing soy isoflavones on serum concentration of cell adhesion molecules: A systematic review and meta-analysis of randomized controlled trials.
Hariri M, Baradaran HR, Gholami A
Complement Ther Med 2021 Sep;61:102764. Epub 2021 Jul 29 doi: 10.1016/j.ctim.2021.102764. PMID: 34333131
Effect of Nigella sativa L. supplementation on inflammatory and oxidative stress indicators: A systematic review and meta-analysis of controlled clinical trials.
Mohit M, Farrokhzad A, Faraji SN, Heidarzadeh-Esfahani N, Kafeshani M
Complement Ther Med 2020 Nov;54:102535. Epub 2020 Aug 9 doi: 10.1016/j.ctim.2020.102535. PMID: 33183658

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