Affiliations: Department of Clinical Immunology, St James's
University Hospital, Beckett St, Leeds LS9 7TF, UK. E-mail:
Philip.wood@leedsth.nhs.uk
Abstract: The primary antibody deficiency syndromes are a rare group of
disorders that can present at any age and for which delay in diagnosis remains
common. Replacement therapy with immunoglobulin in primary antibody
deficiencies increases life expectancy and reduces infection frequency and
severity. Higher doses of immunoglobulin are associated with reduced infection
frequency. Late diagnosis and delayed institution of immunoglobulin replacement
therapy results in increased morbidity with a wide variety of organ-specific
complications and increased mortality. Therapy with immunoglobulin leads to
improvements in overall quality of life, and many of the improvements relate to
reduced infection rates and fear of future infections, strongly suggesting that
the immunoglobulin therapy itself is the major factor in this improvement.
There is limited data on the economic benefits of immunoglobulin therapy, with
the fluctuating costs of immunoglobulin making comparison between different
studies difficult. However, estimates suggest that early intervention with
immunoglobulin replacement compares favourably with prolonged therapy for other
more common chronic diseases.
Keywords: Antibody deficiency, immunoglobulin therapy, common variable immunodeficiency