About this Research Topic

Background

Inborn errors of immunity (IEIs), previously known as primary immunodeficiencies, are characterized by increased susceptibility to infections, autoimmunity, autoinflammation, lymphoproliferation, allergy, and cancer. While each type of IEI individually is a rare disorder, collectively all IEIs are much more than what the general population thinks. With the application of next-generation sequencing technology, an increasing number of IEIs are identified. So far, over 430 IEIs have been reported.

Unlike other rare inherited metabolic disorders for which few treatment modalities are available, IEIs are treatable and curable. Antimicrobials are widely used for the treatment, as well as prophylaxis of various infections. Steroids, immunosuppressants, and anti-cytokine biologics are effective in treating autoimmunity and autoinflammatory conditions. Ig replacement is the mainstay of therapy for patients with antibody deficiencies, and subcutaneous Ig is witnessing a significant increase in application. Hematopoietic stem cell transplantation provides a curative therapy for a wide range of IEIs, and has advanced enormously as a result of improved donor matching, conditioning strategy, supportive care, and graft-versus-host disease prophylaxis. Gene therapy and gene editing hold great promise for treating IEIs and are worthwhile further investigation. With the clarification of underlying pathogenesis, targeted therapies are attractive and have shown their advantages over the conventional treatments for some IEIs. The advances in these treatment modalities lead to a good outcome for a large number of IEIs. On the other hand, due to their rarity, multi-center collaboration is important to gain meaningful insights into the understanding and management of IEIs.

All Original Research, Reviews, Methods, and Perspective articles are welcome. This Research Topic aims to address, but is not limited to, the following themes:
1. Anti-microbial treatment or prophylaxis for IEIs
2. Immunosuppressant and biologics for treating autoimmunity and autoinflammatory conditions
3. Ig replacement, either IVIG or SCIG, for IEIs
4. Hematopoietic stem cell transplantation for IEIs
5. Gene therapy, gene editing for IEIs
6. Targeted therapies for IEIs
7. Novel treatment modalities for IEIs

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