Infection prevention in patients with inborn errors of immunity: Insights from Japan's nationwide study.

Differences in infection prevention methods and their corresponding effects on patients with inborn errors of immunity (IEI) between departments that treat patients with IEI and other departments, such as local hospitals, are not known. We compared the differences in infection prevention methods between low-volume departments (treating fewer than 20 patients with IEI) and high-volume departments (treating 20 or more patients with IEI) using recent nationwide survey data from Japan. There was an uneven distribution of IEI categories between low- and high-volume departments. High-volume departments have set higher target trough levels for immunoglobulin replacement therapy in patients with BTK deficiency and those who have undergone transplantation. Furthermore, sulfamethoxazole-trimethoprim and itraconazole were used more frequently in high-volume departments for patients with hyper-IgE syndrome. Additionally, more patients with common variable immunodeficiency in high-volume departments received inactivated vaccines, whereas low-volume departments used macrolides more frequently than prophylactic antibiotics. Overall, infection prevention management appeared to be relatively similar in low- and high-volume departments. However, there were some notable differences in the specific practices. The latest and most optimal infection control methods must be shared with every department where patients with IEI are treated.