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Decreased circulating C1q concentration

MedGen UID:
1841603
Concept ID:
C5826513
Finding
HPO: HP:0034601

Definition

Concentration or activity of the C1q protein in the blood circulation below the lower limit of normal. C1q is the first component of the classical pathway and is composed of the C1qA chain, C1qB chain, and C1qC chain, which are encoded by C1qA, C1qB, and C1qC genes. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDecreased circulating C1q concentration

Conditions with this feature

C1Q deficiency 2
MedGen UID:
1841058
Concept ID:
C5830422
Disease or Syndrome
C1q deficiency (C1QD) is a rare autosomal recessive disorder characterized by recurrent skin lesions, chronic infections, and an increased risk of autoimmune diseases, particularly systemic lupus erythematosus (SLE; see 152700) or SLE-like diseases. It has also been associated with chronic glomerulonephritis and renal failure. C1q deficiency presents in 2 different forms, absent C1q protein or presence of a dysfunctional molecule (summary by Topaloglu et al., 1996 and Vassallo et al., 2007). For a discussion of genetic heterogeneity of C1q deficiency, see 613652.
C1Q deficiency 3
MedGen UID:
1841059
Concept ID:
C5830423
Disease or Syndrome
C1q deficiency (C1QD) is a rare autosomal recessive disorder characterized by recurrent skin lesions, chronic infections, and an increased risk of autoimmune diseases, particularly systemic lupus erythematosus (SLE; see 152700) or SLE-like diseases. It has also been associated with chronic glomerulonephritis and renal failure. C1q deficiency presents in 2 different forms, absent C1q protein or presence of a dysfunctional molecule (summary by Topaloglu et al., 1996 and Vassallo et al., 2007). For a discussion of genetic heterogeneity of C1q deficiency, see 613652.

Professional guidelines

PubMed

Fava A, Wagner CA, Guthridge CJ, Kheir J, Macwana S, DeJager W, Gross T, Izmirly P, Belmont HM, Diamond B, Davidson A, Utz PJ, Weisman MH, Magder LS; Accelerating Medicines Partnership Rheumatoid Arthritis and Systemic Lupus Erythematosus (AMP RA/SLE) Network, Guthridge JM, Petri M, Buyon J, James JA
Arthritis Rheumatol 2024 Nov;76(11):1611-1622. Epub 2024 Aug 9 doi: 10.1002/art.42941. PMID: 38962936Free PMC Article
Sjöholm AG, Brun C, Larsen S, Thysell H
Int Arch Allergy Appl Immunol 1983;72(1):9-15. doi: 10.1159/000234833. PMID: 6874103

Recent clinical studies

Etiology

Fava A, Wagner CA, Guthridge CJ, Kheir J, Macwana S, DeJager W, Gross T, Izmirly P, Belmont HM, Diamond B, Davidson A, Utz PJ, Weisman MH, Magder LS; Accelerating Medicines Partnership Rheumatoid Arthritis and Systemic Lupus Erythematosus (AMP RA/SLE) Network, Guthridge JM, Petri M, Buyon J, James JA
Arthritis Rheumatol 2024 Nov;76(11):1611-1622. Epub 2024 Aug 9 doi: 10.1002/art.42941. PMID: 38962936Free PMC Article
Dijkstra DJ, Lokki AI, Gierman LM, Borggreven NV, van der Keur C, Eikmans M, Gelderman KA, Laivuori H; FINNPEC Core Investigator Group, Iversen AC, van der Hoorn MP, Trouw LA
Front Immunol 2022;13:842451. Epub 2022 Mar 31 doi: 10.3389/fimmu.2022.842451. PMID: 35432365Free PMC Article
Achieng AO, Guyah B, Cheng Q, Ong'echa JM, Ouma C, Lambert CG, Perkins DJ
EBioMedicine 2019 Jul;45:278-289. Epub 2019 Jun 27 doi: 10.1016/j.ebiom.2019.06.040. PMID: 31257148Free PMC Article
Zou QM, Li XH, Song RX, Xu NP, Zhang T, Zhang MM, Lin Y, Shi L, Fu J, Cui XD
Pediatr Res 2015 Aug;78(2):205-11. Epub 2015 May 4 doi: 10.1038/pr.2015.81. PMID: 25938736
Okabe N, Fujita K, Takenaka K, Yao T
J Clin Lab Immunol 1986 Feb;19(2):77-81. PMID: 3485720

Diagnosis

Fava A, Wagner CA, Guthridge CJ, Kheir J, Macwana S, DeJager W, Gross T, Izmirly P, Belmont HM, Diamond B, Davidson A, Utz PJ, Weisman MH, Magder LS; Accelerating Medicines Partnership Rheumatoid Arthritis and Systemic Lupus Erythematosus (AMP RA/SLE) Network, Guthridge JM, Petri M, Buyon J, James JA
Arthritis Rheumatol 2024 Nov;76(11):1611-1622. Epub 2024 Aug 9 doi: 10.1002/art.42941. PMID: 38962936Free PMC Article
Ushikubo M, Saito S, Kikuchi J, Takeshita M, Yoshimoto K, Yasuoka H, Yamaoka K, Seki N, Suzuki K, Oshima H, Takeuchi T
Lupus 2021 Jan;30(1):61-69. Epub 2020 Oct 28 doi: 10.1177/0961203320967761. PMID: 33115371
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
Zou QM, Li XH, Song RX, Xu NP, Zhang T, Zhang MM, Lin Y, Shi L, Fu J, Cui XD
Pediatr Res 2015 Aug;78(2):205-11. Epub 2015 May 4 doi: 10.1038/pr.2015.81. PMID: 25938736
Díez JJ, Iglesias P
Eur J Endocrinol 2003 Mar;148(3):293-300. doi: 10.1530/eje.0.1480293. PMID: 12611609

Therapy

Trivyza MS, Stergiopoulou C, Tsakas S, Ntrinias T, Papasotiriou M, Karydis N, Papachristou E, Goumenos DS
Int J Mol Sci 2024 Oct 10;25(20) doi: 10.3390/ijms252010904. PMID: 39456685Free PMC Article
Achieng AO, Guyah B, Cheng Q, Ong'echa JM, Ouma C, Lambert CG, Perkins DJ
EBioMedicine 2019 Jul;45:278-289. Epub 2019 Jun 27 doi: 10.1016/j.ebiom.2019.06.040. PMID: 31257148Free PMC Article
Choi KM, Hwang SY, Hong HC, Choi HY, Yoo HJ, Youn BS, Baik SH, Seo HS
Cardiovasc Diabetol 2014 Jan 13;13:14. doi: 10.1186/1475-2840-13-14. PMID: 24417980Free PMC Article
Blomquist S, Gustafsson V, Manolopoulos T, Pierre L
Perfusion 2009 Jan;24(1):13-7. doi: 10.1177/0267659109106730. PMID: 19567543
Díez JJ, Iglesias P
Eur J Endocrinol 2003 Mar;148(3):293-300. doi: 10.1530/eje.0.1480293. PMID: 12611609

Prognosis

Fava A, Wagner CA, Guthridge CJ, Kheir J, Macwana S, DeJager W, Gross T, Izmirly P, Belmont HM, Diamond B, Davidson A, Utz PJ, Weisman MH, Magder LS; Accelerating Medicines Partnership Rheumatoid Arthritis and Systemic Lupus Erythematosus (AMP RA/SLE) Network, Guthridge JM, Petri M, Buyon J, James JA
Arthritis Rheumatol 2024 Nov;76(11):1611-1622. Epub 2024 Aug 9 doi: 10.1002/art.42941. PMID: 38962936Free PMC Article
Shanaki M, Moradi N, Fadaei R, Zandieh Z, Shabani P, Vatannejad A
PLoS One 2018;13(12):e0208059. Epub 2018 Dec 12 doi: 10.1371/journal.pone.0208059. PMID: 30540803Free PMC Article
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
van Eijk IC, Tushuizen ME, Sturk A, Dijkmans BA, Boers M, Voskuyl AE, Diamant M, Wolbink GJ, Nieuwland R, Nurmohamed MT
Ann Rheum Dis 2010 Jul;69(7):1378-82. Epub 2009 Nov 16 doi: 10.1136/ard.2009.118372. PMID: 19919943
Nezlin R
J Immunol Methods 2000 Apr 3;237(1-2):1-17. doi: 10.1016/s0022-1759(00)00139-3. PMID: 10725447

Clinical prediction guides

Trivyza MS, Stergiopoulou C, Tsakas S, Ntrinias T, Papasotiriou M, Karydis N, Papachristou E, Goumenos DS
Int J Mol Sci 2024 Oct 10;25(20) doi: 10.3390/ijms252010904. PMID: 39456685Free PMC Article
Fava A, Wagner CA, Guthridge CJ, Kheir J, Macwana S, DeJager W, Gross T, Izmirly P, Belmont HM, Diamond B, Davidson A, Utz PJ, Weisman MH, Magder LS; Accelerating Medicines Partnership Rheumatoid Arthritis and Systemic Lupus Erythematosus (AMP RA/SLE) Network, Guthridge JM, Petri M, Buyon J, James JA
Arthritis Rheumatol 2024 Nov;76(11):1611-1622. Epub 2024 Aug 9 doi: 10.1002/art.42941. PMID: 38962936Free PMC Article
Kon M, Tanimura Y
Cytokine 2023 Jan;161:156083. Epub 2022 Nov 7 doi: 10.1016/j.cyto.2022.156083. PMID: 36356496
Dijkstra DJ, Lokki AI, Gierman LM, Borggreven NV, van der Keur C, Eikmans M, Gelderman KA, Laivuori H; FINNPEC Core Investigator Group, Iversen AC, van der Hoorn MP, Trouw LA
Front Immunol 2022;13:842451. Epub 2022 Mar 31 doi: 10.3389/fimmu.2022.842451. PMID: 35432365Free PMC Article
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

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