Abstract
Primary immunodeficiency disorders (PIDs) are a group of genetic defects characterized by abnormalities of one or more components of the immune system. While there have been several advances in diagnosis, management, and research in the field of PIDs, they continue to remain underdiagnosed, especially in the less affluent countries. Despite several limitations and challenges, India has advanced significantly in the field of PIDs in the last few years. In this review, we highlight the progress in the field of PIDs in India over the last 25 years, the difficulties faced by clinicians across the country, the current state of PIDs in India and the future prospects.
Highlights
Primary immunodeficiency disorders (PIDs) are a group of genetic defects characterized by abnormalities of one or more components of the immune system
The International Union of Immunological Societies Expert Committee on classification of PIDs has recently updated the phenotypic classification for PIDs [2]
The following search terms were used to find out the number of PubMed publications in PID in the last 50 years in India: India and Primary immune deficiency; Wiskott–Aldrich syndrome and India; Severe combined immunodeficiency and India; X-linked agammaglobulinemia and India; Chronic granulomatous disease and India; Neutropenia and India; Leucocyte adhesion defect and India; Hyper IgE syndrome and India; Hyper-IgM syndrome and India; Chédiak–Higashi syndrome and India; Hemophagocytic lymphohistiocytosis and India; DOCK-8 deficiency and India; Papillon–Lefevre syndrome and India; Ataxia telangiectasia and India; Mendelian susceptibility to mycobacterial diseases and India; Chronic mucocutaneous candidiasis and India; hematopoietic stem cell transplantation (HSCT) and Primary Immune deficiency and India; Intravenous immunoglobulin and India and Primary immunodeficiency
Summary
Primary immunodeficiency disorders (PIDs) are a group of genetic defects characterized by abnormalities of one or more components of the immune system. More than 300 genetically defined single-gene inborn errors of immunity are recognized as a cause of PID [1]. The International Union of Immunological Societies Expert Committee on classification of PIDs has recently updated the phenotypic classification for PIDs [2]. These diseases may affect innate immunity and/or adaptive immunity, and they result in a wide range of manifestations including, but not limited to, susceptibility to infections, autoimmunity, inflammation, allergy, and malignancy.
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