Treatment of Primary immune deficiency diseases (PIDDs)


Treatment of Primary immune deficiency diseases (PIDDs)


Intravenous or subcutaneous IgG infusions at a dose of 400 to 600 mg/kg every 4 weeks are highly effective in preventing chronic infections in agammaglobulinemic patients. Prophylactic antibiotics are indicated in those with chronic lung disease. Adequate IVIg replacement has markedly reduced the incidence of enteroviral infections, but other complications, such as Crohn-like disease, are difficult to prevent, and progressive neurodegeneration has been observed in a small number of XLA patients without identification of an infectious agent.9