eMedicine Specialties > Pediatrics: General Medicine > Allergy & Immunology

Complement Deficiency: Treatment & Medication

Author: Ruchir Agrawal, MD,, Chief, Allergy and Immunology, Aurora Sheboygan Clinic
Coauthor(s): Vishweswar Chilumkurti, MBBS, MPH, Rehabilitation Consultant, Career Employment Australia; Harumi Jyonouchi, MD, Associate Professor, Division of Pulmonary Allergy/Immunology and Infectious Diseases, Department of Pediatrics, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: May 6, 2009

Treatment

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. 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.4

Consultations

  • Consultation with a physician who specializes in immunodeficiency disorders may be considered.

Medication

Vaccine

Active immunization increases resistance to infection. Vaccines consist of microorganisms or cellular components, which act as antigens. Administration of the vaccine stimulates the production of antibodies with specific protective properties. Meningococcal disease is common among patients with terminal common complement pathway (C3, C5-9) deficiencies. Prevention of meningococcal meningitis is recommended.


Meningococcal vaccine (Menactra, Menomune-A/C/Y/W-135)

Isolated from groups A, C, Y, and W-135. Recommended for all individuals with known complement deficiency and those in close contact with the patient. The immunogenicity and clinical efficacy of serogroups A and C meningococcal vaccines have been well established. This vaccine does not confer any protection against serogroup B. The vaccine induces antibody response for serogroup A in individuals as young as 3 mo, but it is poorly immunogenic for serogroup C in recipients who are younger than 18-24 mo.

Adult

0.5 mL SC

Pediatric

<2 years: Contraindicated
>2 years: Administer as in adults

Coadministration with whole-cell pertussis or whole-cell typhoid vaccines may increase endotoxin content; immunosuppressive drugs may interfere with immune response

Documented hypersensitivity; avoid during course of acute illness; children <2 years; IV/IM/ID administration

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Deficiencies in late complement components (C3, C5-C9); do not administer IV/IM/ID; functional or actual asplenia; persons with laboratory or industrial exposure to N meningitidis aerosols; travelers to and residents of hyperendemic areas such as sub-Saharan Africa
For information concerning geographic areas in which vaccination is recommended, contact Centers for Disease Control and Prevention at (404) 332-4559

Androgenic agents

These agents may be used to prevent attacks associated with angioedema. Synthetic attenuated androgens taken prophylactically increase the serum concentration of C1NH.


Danazol (Danocrine)

Increases levels of C4 component of complement by increasing C1 esterase inhibitor, and thereby reduces attacks associated with angioedema.

Adult

400-600 mg/d PO divided bid/tid

Pediatric

Not established

Decreases insulin requirements and increases effects of anticoagulants; coadministration increases carbamazepine and cyclosporine blood levels

Documented hypersensitivity; seizure disorders; hepatic, renal, or hepatic insufficiency; pregnancy and lactation; conditions influenced by edema; porphyria

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Caution in renal, hepatic, or cardiac insufficiency; seizure disorders; peliosis hepatitis and benign hepatic adenoma have been observed with long-term therapy; thromboembolic events and pseudotumor cerebri; androgenlike effects, including weight gain, acne, hirsutism, edema, hair loss, voice changes, and menstrual disturbance

More on Complement Deficiency

Overview: Complement Deficiency
Differential Diagnoses & Workup: Complement Deficiency
Treatment & Medication: Complement Deficiency
Follow-up: Complement Deficiency
Multimedia: Complement Deficiency
References
Further Reading
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References

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Keywords

complement deficiency, complement system, mannose-binding lectin pathway, MBL pathway, classical pathway, alternative pathway, properdin, factor B, factor D, factor H, factor I, C3 component, late complement components, regulatory proteins, C1 inhibitor, C1INH, primary immunodeficiency, Streptococcus pneumoniae, Haemophilus influenzae, proteinuria, Fanconi syndrome, systemic lupus erythematosus, SLE, atherosclerosis, pneumococcal infection, MBL deficiency, schizophrenia, hemolytic-uremic syndrome, HUS, membranoproliferative glomerulonephritis, rheumatoid arthritis, Neisseria gonorrhoeae, Neisseria meningitidis, angioedema, collagen vascular disorders, cystic fibrosis, CF, treatment, diagnosis

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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.

Medical Editor

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.

Pharmacy Editor

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

Managing Editor

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.

CME Editor

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

 
 
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