Clinical implications of C6 complement component deficiency
Christine K Rauscher1, Merritt L Fajt1, Jodie Bryk2
1From the Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania; and.
Insights
Terminal complement component deficiencies increase the risk of neisserial infections. C6 deficiency, the most common form, can manifest later in life with Meningitis infections.
Area of Science:
- Immunology
- Infectious Diseases
Background:
- Terminal complement component deficiencies are established risk factors for severe neisserial infections.
- Understanding these deficiencies is crucial for managing recurrent infections.
Observation:
- A case of C6 deficiency in a 56-year-old patient with meningitis and chronic rash is presented.
- This case highlights the varied clinical presentations of terminal complement deficiencies.
Findings:
- C6 deficiency is the most prevalent terminal complement component deficiency.
- Late-onset Neisseria meningitidis infections can occur in individuals with C6 deficiency.
Implications:
- Screening for terminal complement component deficiency using CH50 assays is recommended.
- This review provides clinical pearls for allergists/immunologists managing these patients.
Abstract:
Background: Terminal complement component deficiencies are risk factors for neisserial infections. Objective: To review the clinical characteristics, the diagnosis and the management of patients with a terminal complement component deficiency. Methods: Pertinent articles were selected and reviewed in relation to a case presentation of C6 deficiency. Results: A case of a 56-year old patient with a history of meningitis, chronic rash, and C6 deficiency was presented, followed by discussion of clinical characteristics, diagnosis, and management of terminal complement component deficiencies. Clinical pearls and pitfalls were reviewed for the practicing allergist/immunologist and fellow-in-training. Conclusion: C6 deficiency is the most common terminal complement component deficiency and can present later in age with N. meningitidis infections. Patients can be screened for terminal complement component deficiency by checking CH50.
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