Immunoglobulin replacement therapy in the Czech Republic: end-of-year 2020 data from the National registry of primary immunodeficiencies
Abstract
Immunoglobulin replacement therapy is the most frequently used therapeutic approach in patients with congenital defects of antibody production, which represent the largest group of patients among primary immunodeficiency disorders. The National registry of primary immunodeficiency disorders serves as a useful tool for nationwide monitoring of immunoglobulin replacement therapy consumption in this patient.
Out of the total number of 1029 patients with referred in registry, a total of 501 (48.7%) of them were treated with immunoglobulin replacement therapy. The majority of patients belonged to the group of patients with antibody deficiency disorders (479; 95.6%), other patients suffered from dia- gnoses of other well-defined immunodeficiencies (16; 3.2%), combined immunodeficiencies (5; 1.0%) and defects of the complement system (1; 0.2%).
Most patients were treated with conventional subcutaneous (212 patients; 42.3%), intravenous (179 patients; 35.7%), and finally hyaluronidase-facilitated subcutaneous immunoglobulin administration (98 patients; 19.6%). In 12 patients (2.4%), intramuscular administration was reported.
The average dose of was below the recommended 0.40 g/kg/month (intravenous: 0.32 g/kg/month, conventional subcutaneous 0.27 g/kg/month, facilitated subcutaneous: 0.37 g/kg/month). The averaged trough IgG levels were 6 g/l for all routes of administration.
The immunoglobulin replacement therapy of patients with primary immunodeficiency in the Czech Republic did not reach the level of developed countries. To maintain the quality of care for immunological patients, despite the worldwide increasing consumption of these drugs due to the expansion of treatment indications and limited human resources for their production, it is important to obtain valid data on usage of these drugs in our country.