RESUMO
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.
Assuntos
Envelhecimento/fisiologia , Complemento C6/deficiência , Complemento C6/genética , Doenças da Deficiência Hereditária de Complemento/diagnóstico , Meningite Meningocócica/diagnóstico , Vacinas Meningocócicas/imunologia , Neisseria meningitidis/fisiologia , Antibioticoprofilaxia , Ensaio de Atividade Hemolítica de Complemento , Feminino , Fibronectinas/análise , Doenças da Deficiência Hereditária de Complemento/complicações , Humanos , Meningite Meningocócica/etiologia , Meningite Meningocócica/prevenção & controle , Pessoa de Meia-Idade , Proteínas Recombinantes/análiseRESUMO
WHAT IS KNOWN AND OBJECTIVE: Tumour necrosis factor-α-blocking agents potentially cause vasculitis. However, no study has reported on the association between hypocomplementemic urticarial vasculitis (HUV) and certolizumab pegol (CZP) usage. CASE DESCRIPTION: We present the first case of HUV development during CZP treatment for rheumatoid arthritis. Hypocomplementemic urticarial vasculitis improved after CZP was discontinued and the dose of oral prednisolone was increased. WHAT IS NEW AND CONCLUSION: Clinicians should be aware about the potential development of HUV during CZP treatment, which is presumed to be safe considering its unique structural characteristics that differ from those of other tumour necrosis factor-α-blocking agents.