Complement Deficiency Follow-up

  • Author: Ruchir Agrawal, MD; Chief Editor: Russell W Steele, MD   more...
 
Updated: May 6, 2009
 

Deterrence/Prevention

  • Prevention of infections and immediate treatment are important aspects of treatment in patients with complement deficiency. Treat acute infections with appropriate antibiotics and prevent with vaccination.
  • All routine vaccines are recommended. Meningococcal, pneumococcal, and haemophilus vaccinations are recommended for patients and those who are in close contact with them.
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Prognosis

  • Properdin deficiency is associated with a high mortality rate.
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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  Rehabilitation Consultant, Career Employment Australia

Disclosure: Nothing to disclose.

Harumi Jyonouchi, MD  Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-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 Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine

Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

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

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: None None None

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. [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].

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

  6. 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].

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

  8. 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].

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

  10. 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].

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

  12. 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].

  13. 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].

  14. 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].

  15. 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].

  16. 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].

  17. 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].

  18. 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].

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

  20. 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].

  21. 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].

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

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

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

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

  26. 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].

  27. 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].

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

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

  30. 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].

  31. 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].

  32. 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].

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

  34. 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].

  35. 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].

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

 
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Scheme showing the cascade of events during the activation of the complement system.
 
 
 
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