Further Outpatient Care

Patients with a known complement deficiency should be screened for glomerular or immune complex disease. Obtain urinalysis results to check for proteinuria and rheumatologic serology results to screen for SLE.

Further Inpatient Care

Serious infectious states warrant hospitalization for treatment.

Inpatient treatment is not necessarily needed to screen for complement deficiencies if the patient is asymptomatic.

Inpatient & Outpatient Medications

Cephalosporins (third- or fourth-generation) are needed for treatment of meningeal infection.

Deterrence/Prevention

Administration of the multivalent meningococcal vaccine is recommended in patients with known complement deficiency, especially those patients deficient in the MAC proteins. Similarly, administration of the pneumococcal vaccine and the Haemophilus influenzae vaccine also may provide protection against these encapsulated organisms.

Complications

Complications of complement deficiencies can be serious; severe CNS damage and death from meningitis are among the worst possible adverse outcomes.

Prognosis

In general, the prognosis for patients with C3 deficiencies is poorer than that of individuals with other complement deficiencies. Patients may have severe, recurrent episodes of pyogenic infection beginning when as young as a few months. Many can die from sepsis early in life.

Patients with a deficiency of one of the early components of the classical pathway (C1, C4, C2) are at high risk for autoimmune disease but at lower risk for overwhelming sepsis with pyogenic infections.

Deficiency of a MAC component (C5, C6, C7, C8) or of properdin increases the risk for recurrent infections caused by Neisseria organisms.

Mannan-binding lectin (MBL) deficiency has been linked to an increased frequency of pyogenic infections and sepsis, especially in neonates and children.

Patient Education

Patients with an identified complement deficiency should be counseled regarding possible complications and risks associated with this deficiency.

Family members should be screened for complement deficiencies and counseled regarding possible risks.

Questions

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Media Gallery

Complement pathways and deficiencies.

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Coauthor(s)

R Krishna Chaganti, MD Assistant Clinical Professor, Division of Rheumatology, UCSF

R Krishna Chaganti, MD is a member of the following medical societies: American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Michael A Kaliner, MD Clinical Professor of Medicine, George Washington University School of Medicine; Medical Director, Institute for Asthma and Allergy

Michael A Kaliner, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Clinical Investigation, American Thoracic Society, Association of American Physicians

Disclosure: Nothing to disclose.

Acknowledgements

Margaret R Donohoe, MD Consulting Staff, Department of Allergy and Immunology, Albemarle Hospital

Disclosure: Nothing to disclose.

Samuel R Marney, Jr, MD Director, Associate Professor, Department of Internal Medicine, Division of Allergy and Immunology, Vanderbilt University School of Medicine

Samuel R Marney, Jr, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, and Tennessee Medical Association

Disclosure: Nothing to disclose.

Darrilyn Moyer, MD Associate Program Director, Associate Professor, Department of Internal Medicine, Temple University School of Medicine

Disclosure: Nothing to disclose.