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Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency)

¹ Department of Immunology, Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne NE1 4LP, UK
² Department of Paediatrics, Newcastle upon Tyne Hospitals NHS Trust

Correspondence to:
Dr A Fay, Department of Immunology, Royal Victoria Infirmary, Newcastle upon Tyne NE1 4LP, UK;
Fiona.Campbell{at}nuth.northy.nhs.uk
ABSTRACT
Hereditary angio-oedema is characterised by recurrent swellings in any part of the body and also by recurrent attacks of severe abdominal pain. The disease is inherited in an autosomal dominant manner but up to 25% of cases can occur as a spontaneous mutation. Attacks of swelling can be precipitated by trauma, certain drugs, and emotional stress. Treatment usually involves a combination of prophylaxis, using androgens or antifibrolytic drugs, and replacement with C'1 esterase inhibitor concentrate for acute attacks and before surgery or other traumatic procedures.

Keywords: hereditary angio-oedema; C`1 inhibitor protein; complement activation; treatment

Abbreviations: FFP, fesh frozen plasma; HAE, hereditary angio-oedema; HCV, hepatitis C virus; HGV, hepatitis G virus

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