Complement Deficiency Treatment & Management

  • Author: Ruchir Agrawal, MD; Chief Editor: Russell W Steele, MD   more...
 
Updated: Mar 22, 2012
 

Medical Care

  • 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.[4] 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.[5, 6]
Next

Consultations

  • Consultation with a physician who specializes in immunodeficiency disorders may be considered.
Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Ruchir Agrawal, MD  Chief, Allergy and Immunology, Aurora Sheboygan Clinic

Ruchir Agrawal, MD, is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American College of Allergy, Asthma and Immunology, and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Vishweswar Chilumkurti, MBBS, MPH, MHA  Consultant, CEA, Australia

Vishweswar Chilumkurti, MBBS, MPH, MHA is a member of the following medical societies: Indian Medical Association and Medical Council of India

Disclosure: Nothing to disclose.

Harumi Jyonouchi, MD  Associate Professor, Division of Pulmonary, Allergy/Immunology, and Infectious Diseases, Department of Pediatrics, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Ann O'Neill Shigeoka, MD †  Former Clinical Associate Professor, Department of Pediatrics, Division of Immunology-Rheumatology, University of Utah School of Medicine

Ann O'Neill Shigeoka, MD † is a member of the following medical societies: American Federation for Medical Research, Clinical Immunology Society, Pediatric Infectious Diseases Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John Wilson Georgitis, MD  Consulting Staff, Lafayette Allergy Services

John Wilson Georgitis, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American College of Chest Physicians, American Lung Association, American Medical Writers Association, and American Thoracic Society

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville School of Medicine

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Russell W Steele, MD  Head, Division of Pediatric Infectious Diseases, Ochsner Children's Health Center; Clinical Professor, Department of Pediatrics, Tulane University School of Medicine

Russell W Steele, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Microbiology, Infectious Diseases Society of America, Louisiana State Medical Society, Pediatric Infectious Diseases Society, Society for Pediatric Research, and Southern Medical Association

Disclosure: Nothing to disclose.

References

  1. 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. Aug 1999;104(4):431-7. [Medline].

  2. Tedesco F. Inherited complement deficiencies and bacterial infections. Vaccine. Dec 30 2008;26 Suppl 8:I3-8. [Medline].

  3. Janzi M, Sjoberg R, Wan J, Fischler B, et al. Screening for C3 deficiency in newborns using microarrays. PLoS ONE. 2009;4(4):e5321. [Medline].

  4. 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. Mar 2012;167(3):472-8. [Medline].

  5. [Best Evidence] 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. Dec 2008;153(6):764-70. [Medline].

  6. 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. Feb 26 2012;[Medline].

  7. AAP 2000 Red Book. Report of the Committee on Infectious Diseases. 25th ed. 2000:56-7.

  8. Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. May 2005;94(5 Suppl 1):S1-63. [Medline].

  9. Carroll MC. CD21/CD35 in B cell activation. Semin Immunol. Aug 1998;10(4):279-86. [Medline].

  10. 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. Feb 1997;99(2):194-6. [Medline].

  11. Cohen J. Meningococcal disease as a model to evaluate novel anti-sepsis strategies. Crit Care Med. Sep 2000;28(9 Suppl):S64-7. [Medline].

  12. 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. Mar 2000;35(3):401-7. [Medline].

  13. Frank MM. Complement deficiencies. Pediatr Clin North Am. Dec 2000;47(6):1339-54. [Medline].

  14. Jackson LA, Schuchat A, Reeves MW, Wenger JD. Serogroup C meningococcal outbreaks in the United States. An emerging threat. JAMA. Feb 1 1995;273(5):383-9. [Medline].

  15. 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].

  16. 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. Dec 1999;110:190-3. [Medline].

  17. 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. Apr 2006;117(4):904-8. [Medline].

  18. 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].

  19. 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. Mar 2001;37(3):490-8. [Medline].

  20. 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. Apr 2006;117(4):883-96. [Medline].

  21. Ratnoff WD. Inherited deficiencies of complement in rheumatic diseases. Rheum Dis Clin North Am. Feb 1996;22(1):75-94. [Medline].

  22. Ross SC, Densen P. Complement deficiency states and infection: epidemiology, pathogenesis and consequences of neisserial and other infections in an immune deficiency. Medicine (Baltimore). Sep 1984;63(5):243-73. [Medline].

  23. Rougier N, Kazatchkine MD, Rougier JP, et al. Human complement factor H deficiency associated with hemolytic uremic syndrome. J Am Soc Nephrol. Dec 1998;9(12):2318-26. [Medline].

  24. Salzman MB, Rubin LG. Meningococcemia. Infect Dis Clin North Am. Dec 1996;10(4):709-25. [Medline].

  25. Sjoholm AG, Jonsson G, Braconier JH, et al. Complement deficiency and disease: an update. Mol Immunol. Jan 2006;43(1-2):78-85. [Medline].

  26. Sturfelt G, Truedsson L. Complement and its breakdown products in SLE. Rheumatology (Oxford). Oct 2005;44(10):1227-32. [Medline].

  27. Sullivan KE. Genetics of systemic lupus erythematosus. Clinical implications. Rheum Dis Clin North Am. May 2000;26(2):229-56, v-vi. [Medline].

  28. Ten RM, Carmona EM, Babovic-Vuksanovic D, Katzmann JA. Mannose-binding lectin deficiency associated with neutrophil chemotactic unresponsiveness to C5a. J Allergy Clin Immunol. Aug 1999;104(2 Pt 1):419-24. [Medline].

  29. 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].

  30. Walport MJ. Complement. First of two parts. N Engl J Med. Apr 5 2001;344(14):1058-66. [Medline].

  31. Walport MJ. Complement. Second of two parts. N Engl J Med. Apr 12 2001;344(15):1140-4. [Medline].

  32. Waytes AT, Rosen FS, Frank MM. Treatment of hereditary angioedema with a vapor-heated C1 inhibitor concentrate. N Engl J Med. Jun 20 1996;334(25):1630-4. [Medline].

  33. Weiss SJ, Ahmed AE, Bonagura VR. Complement factor D deficiency in an infant first seen with pneumococcal neonatal sepsis. J Allergy Clin Immunol. Dec 1998;102(6 Pt 1):1043-4. [Medline].

  34. Wen L, Atkinson JP, Giclas PC. Clinical and laboratory evaluation of complement deficiency. J Allergy Clin Immunol. Apr 2004;113(4):585-93; quiz 594. [Medline].

  35. West CD, McAdams AJ. The alternative pathway C3 convertase and glomerular deposits. Pediatr Nephrol. Jun 1999;13(5):448-53. [Medline].

  36. Whaley K, Ruddy S. Modulation of C3b hemolytic activity by a plasma protein distinct from C3b inactivator. Science. Sep 10 1976;193(4257):1011-3. [Medline].

  37. 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. Jun 2006;117(6):1344-50. [Medline].

  38. Williams DG. C3 nephritic factor and mesangiocapillary glomerulonephritis. Pediatr Nephrol. Feb 1997;11(1):96-8. [Medline].

 
Previous
Next
 
Scheme showing the cascade of events during the activation of the complement system.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.