Complement deficiencies

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

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16. Schröder-Braunstein J, Kirschfink M. Complement deficiencies and dysregulation: Pathophysiological consequences, modern analysis, and clinical management. Mol Immunol. 2019 Oct;114:299-311. Abstract

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21. Noris M, Remuzzi G. Overview of complement activation and regulation. Semin Nephrol. 2013 Nov;33(6):479-92.Full text  Abstract

22. 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

23. Gompels MM, Lock RJ, Albinun M, et al. C1 inhibitor deficiency: consensus document. Clin Exp Immunol. 2005 Mar;139(3):379-94.Full text  Abstract

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26. Walport MJ. Complement: second of two parts. N Engl J Med. 2001 Apr 12;344(15):1140-4. Abstract

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29. Leggiadro RJ, Winkelstein JA. Prevalence of complement deficiencies in children with systemic meningococcal infections. Pediatr Infect Dis J. 1987 Jan;6(1):75-6.Full text  Abstract

30. Quillin SJ, Seifert HS. Neisseria gonorrhoeae host adaptation and pathogenesis. Nat Rev Microbiol. 2018 Apr;16(4):226-40.Full text  Abstract

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32. Levy JH, Freiberger DJ, Roback J. Hereditary angioedema: current and emerging treatment options. Anesth Analg. 2010 May 1;110(5):1271-80.Full text  Abstract

33. Caccia S, Suffritti C, Cicardi M. Pathophysiology of hereditary angioedema. Pediatr Allergy Immunol Pulmonol. 2014 Dec 1;27(4):159-63.Full text  Abstract

34. Zuraw BL, Bernstein JA, Lang DM, et al. A focused parameter update: hereditary angioedema, acquired C1 inhibitor deficiency, and angiotensin-converting enzyme inhibitor-associated angioedema. J Allergy Clin Immunol. 2013 Jun;131(6):1491-3.Full text  Abstract

35. Breitbart SI, Bielory L, Breitbart SI, et al. Acquired angioedema: Autoantibody associations and C1q utility as a diagnostic tool. Allergy Asthma Proc. 2010 Sep-Oct;31(5):428-34. Abstract

36. Bork K, Staubach-Renz P, Hardt J. Angioedema due to acquired C1-inhibitor deficiency: spectrum and treatment with C1-inhibitor concentrate. Orphanet J Rare Dis. 2019 Mar 13;14(1):65.Full text  Abstract

37. Bork K, Gül D, Hardt J, et al. Hereditary angioedema with normal C1 inhibitor: clinical symptoms and course. Am J Med. 2007 Nov;120(11):987-92. Abstract

38. Veronez CL, Csuka D, Sheikh FR, et al. The expanding spectrum of mutations in hereditary angioedema. J Allergy Clin Immunol Pract. 2021 Jun;9(6):2229-34. Abstract

39. 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

40. Macedo AC, Isaac L. Systemic lupus erythematosus and deficiencies of early components of the complement classical pathway. Front Immunol. 2016 Feb 24;7:55.Full text  Abstract

41. Trendelenburg M. Autoantibodies against complement component C1q in systemic lupus erythematosus. Clin Transl Immunology. 2021 Apr 29;10(4):e1279.Full text  Abstract

42. Carroll MC. A protective role for innate immunity in systemic lupus erythematosus. Nat Rev Immunol. 2004 Oct;4(10):825-31. Abstract

43. Weinstein A, Alexander RV, Zack DJ. A review of complement activation in SLE. Curr Rheumatol Rep. 2021 Feb 10;23(3):16.Full text  Abstract

44. Lewis LA, Ram S. Meningococcal disease and the complement system. Virulence. 2014 Jan 1;5(1):98-126.Full text  Abstract

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46. Centers for Disease Control and Prevention. Adult immunization schedule by age: recommendations for ages 19 years or older, United States, 2024. Nov 2023 [internet publication].Full text

47. Cook HT. Complement and kidney disease. Curr Opin Nephrol Hypertens. 2013 May;22(3):295-301. Abstract

48. Noris M, Remuzzi G. Genetics of immune-mediated glomerular diseases: focus on complement. Semin Nephrol. 2017 Sep;37(5):447-63. Abstract

49. Willows J, Brown M, Sheerin NS. The role of complement in kidney disease. Clin Med (Lond). 2020 Mar;20(2):156-60.Full text  Abstract

50. Smith RJH, Appel GB, Blom AM, et al. C3 glomerulopathy - understanding a rare complement-driven renal disease. Nat Rev Nephrol. 2019 Mar;15(3):129-43.Full text  Abstract

51. Cook HT. C3 glomerulopathy. F1000Res. 2017 Mar 10;6:248.Full text  Abstract

52. Corvillo F, Okrój M, Nozal P, et al. Nephritic factors: an overview of classification, diagnostic tools and clinical associations. Front Immunol. 2019 Apr 24;10:886.Full text  Abstract

53. Jokiranta TS. HUS and atypical HUS. Blood. 2017 May 25;129(21):2847-56.Full text  Abstract

54. Fritsche LG, Igl W, Bailey JN, et al. A large genome-wide association study of age-related macular degeneration highlights contributions of rare and common variants. Nat Genet. 2016 Feb;48(2):134-43.Full text  Abstract

55. Hill A, DeZern AE, Kinoshita T, et al. Paroxysmal nocturnal haemoglobinuria. Nat Rev Dis Primers. 2017 May 18;3:17028. Abstract

56. Gorevic PD. Rheumatoid factor, complement, and mixed cryoglobulinemia. Clin Dev Immunol. 2012;2012:439018.Full text  Abstract

57. Levy Y, George J, Yona E, et al. Partial lipodystrophy, mesangiocapillary glomerulonephritis, and complement dysregulation. An autoimmune phenomenon. Immunol Res. 1998 Aug;18(1):55-60. Abstract

58. Prohászka Z, Nilsson B, Frazer-Abel A, et al. Complement analysis 2016: clinical indications, laboratory diagnostics and quality control. Immunobiology. 2016 Nov;221(11):1247-58. Abstract

59. Ling M, Murali M. Analysis of the complement system in the clinical immunology laboratory. Clin Lab Med. 2019 Dec;39(4):579-90. Abstract

60. Frazer-Abel A, Kirschfink M, Prohászka Z. Expanding horizons in complement analysis and quality control. Front Immunol. 2021 Aug 9;12:697313.Full text  Abstract

61. Seelen MA, Roos A, Wieslander J, et al. Functional analysis of the classical, alternative, and MBL pathways of the complement system: standardization and validation of a simple ELISA. J Immunol Methods. 2005 Jan;296(1-2):187-98. Abstract

62. Wehling C, Amon O, Bommer M, et al. Monitoring of complement activation biomarkers and eculizumab in complement-mediated renal disorders. Clin Exp Immunol. 2017 Feb;187(2):304-15.Full text  Abstract

63. Fakhouri F, Frémeaux-Bacchi V. Thrombotic microangiopathy in aHUS and beyond: clinical clues from complement genetics. Nat Rev Nephrol. 2021 Aug;17(8):543-53. Abstract

64. Germenis AE, Margaglione M, Pesquero JB, et al. International consensus on the use of genetics in the management of hereditary angioedema. J Allergy Clin Immunol Pract. 2020 Mar;8(3):901-11. Abstract

65. Licht C, Weyersburg A, Heinen S, et al. Successful plasma therapy for atypical hemolytic uremic syndrome caused by factor H deficiency owing to a novel mutation in the complement cofactor protein domain 15. Am J Kidney Dis. 2005 Feb;45(2):415-21. Abstract

66. Ricklin D, Mastellos DC, Reis ES, et al. The renaissance of complement therapeutics. Nat Rev Nephrol. 2018 Jan;14(1):26-47.Full text  Abstract

67. Fakhouri F, Frémeaux-Bacchi V, Loirat C. Atypical hemolytic uremic syndrome: from the rediscovery of complement to targeted therapy. Eur J Intern Med. 2013 Sep;24(6):492-5. Abstract

68. Mastellos DC, Ricklin D, Lambris JD. Clinical promise of next-generation complement therapeutics. Nat Rev Drug Discov. 2019 Sep;18(9):707-29.Full text  Abstract

69. Caballero T. Treatment of hereditary angioedema. J Investig Allergol Clin Immunol. 2021 Feb;31(1):1-16.Full text  Abstract

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71. Caballero T, Farkas H, Bouillet L, et al. International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency. J Allergy Clin Immunol. 2012 Feb;129(2):308-20.Full text  Abstract

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