Primary Immunodeficiency Disease in Malaysia: Where Are We Now?

Abstract

Primary Immunodeficiency Diseases (PIDs), also called Inborn Errors of Immunity (IEI), are a group of diseases caused by a single gene defect affecting the differentiation or development of the immune system. These diseases usually manifest with increased susceptibility to infections, autoimmunity, autoinflammatory diseases, allergy, bone marrow failure, and/or malignancy. The PID Life Index is a unified continuum of PID care aimed at reducing inter-country gaps and improving patients’ care outcomes. In Malaysia, there has been significant progress in PID care in close to half a century since the first case of PID was discovered. On the other hand, there are bottlenecks facing the progress in PID care in Malaysia. This review was written to assess PID care in Malaysia based on the six components of the PID Life Index.