Complement Deficiency Treatment & Management
- Author: Ruchir Agrawal, MD; Chief Editor: Russell W Steele, MD more...
See the list below:
- No specific treatment is available for genetically acquired complement deficiencies; however, acute attacks of hereditary angioedema (HAE), C1INH deficiency, have been successfully treated with infusion of vapor-heated C1 esterase inhibitor. Androgen therapy can be used to prevent HAE attacks. These treatments are recommended only in adults. A study in the Netherlands indicated efficacy of self-administration of plasma-derived C1INH concentrate for prevention and treatment of angioedema attacks in patients with C1INH deficiency.
- Only supportive therapy is available for other complement deficiencies. Fresh frozen plasma is used for emergent replacement of complement components.
- Genes have been cloned for individual component deficiencies. Therefore, gene therapy may be a choice in the future.
- All routine vaccines are recommended in complement deficiency.
- Meningococcal vaccine is recommended for children with early or terminal complement component or properdin deficiencies.
- Pneumococcal vaccine is recommended for deficiency of early components. The effects of influenza plus pneumococcal conjugate vaccination in preventing respiratory tract infections was recently studied.
See the list below:
- Consultation with a physician who specializes in immunodeficiency disorders may be considered.
Clarke EV, Weist BM, Walsh CM, Tenner AJ. Complement protein C1q bound to apoptotic cells suppresses human macrophage and dendritic cell-mediated Th17 and Th1 T cell subset proliferation. J Leukoc Biol. 2014 Nov 7. [Medline].
Ghannam A, Fauquert JL, Thomas C, Kemper C, Drouet C. Human complement C3 deficiency: Th1 induction requires T cell-derived complement C3a and CD46 activation. Mol Immunol. 2014 Mar. 58(1):98-107. [Medline].
Garred P, Pressler T, Madsen HO, et al. Association of mannose-binding lectin gene heterogeneity with severity of lung disease and survival in cystic fibrosis. J Clin Invest. 1999 Aug. 104(4):431-7. [Medline].
Tedesco F. Inherited complement deficiencies and bacterial infections. Vaccine. 2008 Dec 30. 26 Suppl 8:I3-8. [Medline].
Daures M, John M, Balter CV, Simon O, Barguil Y, Missotte I, et al. Relationships Between Clinico-Epidemiological Patterns of Invasive Meningococcal Infections and Complement Deficiencies in French South Pacific Islands (New Caledonia). J Clin Immunol. 2014 Oct 29. [Medline].
AAP 2000 Red Book. Report of the Committee on Infectious Diseases. 25th ed. 2000. 56-7.
Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005 May. 94(5 Suppl 1):S1-63. [Medline].
Carroll MC. CD21/CD35 in B cell activation. Semin Immunol. 1998 Aug. 10(4):279-86. [Medline].
Cicardi M, Castelli R, Zingale LC, Agostoni A. Side effects of long-term prophylaxis with attenuated androgens in hereditary angioedema: comparison of treated and untreated patients. J Allergy Clin Immunol. 1997 Feb. 99(2):194-6. [Medline].
Cohen J. Meningococcal disease as a model to evaluate novel anti-sepsis strategies. Crit Care Med. 2000 Sep. 28(9 Suppl):S64-7. [Medline].
Endo M, Ohi H, Ohsawa I, et al. Complement activation through the lectin pathway in patients with Henoch-Schonlein purpura nephritis. Am J Kidney Dis. 2000 Mar. 35(3):401-7. [Medline].
Frank MM. Complement deficiencies. Pediatr Clin North Am. 2000 Dec. 47(6):1339-54. [Medline].
Jackson LA, Schuchat A, Reeves MW, Wenger JD. Serogroup C meningococcal outbreaks in the United States. An emerging threat. JAMA. 1995 Feb 1. 273(5):383-9. [Medline].
Jansen AG, Sanders EA, Hoes AW, van Loon AM, Hak E. Effects of influenza plus pneumococcal conjugate vaccination versus influenza vaccination alone in preventing respiratory tract infections in children: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2008 Dec. 153(6):764-70. [Medline].
Kainulainen L, Peltola V, Seppänen M, Viander M, He Q, Lokki ML, et al. C4A deficiency in children and adolescents with recurrent respiratory infections. Hum Immunol. 2012 Feb 26. [Medline].
Kang HJ, Kim HS, Lee YK, et al. High incidence of complement C9 deficiency in Koreans. Ann Clin Lab Sci. 2005. 35(2):144-8. [Medline].
Kumar A, Gupta R, Varghese T, et al. Anti-C1q antibody as a marker of disease activity in systemic lupus erythematosus. Indian J Med Res. 1999 Dec. 110:190-3. [Medline].
Levi M, Choi G, Picavet C, Hack CE. Self-administration of C1-inhibitor concentrate in patients with hereditary or acquired angioedema caused by C1-inhibitor deficiency. J Allergy Clin Immunol. 2006 Apr. 117(4):904-8. [Medline].
Manderson AP, Botto M, Walport MJ. The role of complement in the development of systemic lupus erythematosus. Annu Rev Immunol. 2004. 22:431-56. [Medline].
Momeni N, Bergquist J, Brudin L, Behnia F, Sivberg B, Joghataei MT. A novel blood-based biomarker for detection of autism spectrum disorders. Transl Psychiatry. 2012. 2:e91. [Medline].
Moroni G, Trendelenburg M, Del Papa N, et al. Anti-C1q antibodies may help in diagnosing a renal flare in lupus nephritis. Am J Kidney Dis. 2001 Mar. 37(3):490-8. [Medline].
Notarangelo L, Casanova JL, Conley ME, et al. Primary immunodeficiency diseases: an update from the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee Meeting in Budapest, 2005. J Allergy Clin Immunol. 2006 Apr. 117(4):883-96. [Medline].
Ratnoff WD. Inherited deficiencies of complement in rheumatic diseases. Rheum Dis Clin North Am. 1996 Feb. 22(1):75-94. [Medline].
Ross SC, Densen P. Complement deficiency states and infection: epidemiology, pathogenesis and consequences of neisserial and other infections in an immune deficiency. Medicine (Baltimore). 1984 Sep. 63(5):243-73. [Medline].
Rougier N, Kazatchkine MD, Rougier JP, et al. Human complement factor H deficiency associated with hemolytic uremic syndrome. J Am Soc Nephrol. 1998 Dec. 9(12):2318-26. [Medline].
Salzman MB, Rubin LG. Meningococcemia. Infect Dis Clin North Am. 1996 Dec. 10(4):709-25. [Medline].
Sjoholm AG, Jonsson G, Braconier JH, et al. Complement deficiency and disease: an update. Mol Immunol. 2006 Jan. 43(1-2):78-85. [Medline].
Sturfelt G, Truedsson L. Complement and its breakdown products in SLE. Rheumatology (Oxford). 2005 Oct. 44(10):1227-32. [Medline].
Sullivan KE. Genetics of systemic lupus erythematosus. Clinical implications. Rheum Dis Clin North Am. 2000 May. 26(2):229-56, v-vi. [Medline].
Ten RM, Carmona EM, Babovic-Vuksanovic D, Katzmann JA. Mannose-binding lectin deficiency associated with neutrophil chemotactic unresponsiveness to C5a. J Allergy Clin Immunol. 1999 Aug. 104(2 Pt 1):419-24. [Medline].
U. S. Department of Health and Human Services. Control and Prevention of Meningococcal Disease and Control and Prevention of Serogroup C Meningococcal Disease: Evaluation and Mangement of Suspected Outbreaks. Morbidity and Mortality Weekly Report. February 14, 1997. 46:[Full Text].
van Geffen M, Cugno M, Lap P, Loof A, Cicardi M, van Heerde W. Alterations of coagulation and fibrinolysis in patients with angioedema due to C1-inhibitor deficiency. Clin Exp Immunol. 2012 Mar. 167(3):472-8. [Medline].
Walport MJ. Complement. First of two parts. N Engl J Med. 2001 Apr 5. 344(14):1058-66. [Medline].
Walport MJ. Complement. Second of two parts. N Engl J Med. 2001 Apr 12. 344(15):1140-4. [Medline].
Waytes AT, Rosen FS, Frank MM. Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate. N Engl J Med. 1996 Jun 20. 334(25):1630-4. [Medline].
Weiss SJ, Ahmed AE, Bonagura VR. Complement factor D deficiency in an infant first seen with pneumococcal neonatal sepsis. J Allergy Clin Immunol. 1998 Dec. 102(6 Pt 1):1043-4. [Medline].
Wen L, Atkinson JP, Giclas PC. Clinical and laboratory evaluation of complement deficiency. J Allergy Clin Immunol. 2004 Apr. 113(4):585-93; quiz 594. [Medline].
West CD, McAdams AJ. The alternative pathway C3 convertase and glomerular deposits. Pediatr Nephrol. 1999 Jun. 13(5):448-53. [Medline].
Whaley K, Ruddy S. Modulation of C3b hemolytic activity by a plasma protein distinct from C3b inactivator. Science. 1976 Sep 10. 193(4257):1011-3. [Medline].
Wiertsema SP, Herpers BL, Veenhoven RH, et al. Functional polymorphisms in the mannan-binding lectin 2 gene: effect on MBL levels and otitis media. J Allergy Clin Immunol. 2006 Jun. 117(6):1344-50. [Medline].
Williams DG. C3 nephritic factor and mesangiocapillary glomerulonephritis. Pediatr Nephrol. 1997 Feb. 11(1):96-8. [Medline].