Open Access7 th International Immunoglobulin Conference: Immunodeficiencies
Author(s) -
Schmidt R. E.,
Ochs H. D.
Publication year - 2014
Publication title -
clinical & experimental immunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.329
H-Index - 135
eISSN - 1365-2249
pISSN - 0009-9104
DOI - 10.1111/cei.12490
Subject(s) - medicine , immunosuppression , common variable immunodeficiency , primary immunodeficiency , thymectomy , immunodeficiency , immunology , antibody , transplantation , intensive care medicine , pediatrics , immune system , myasthenia gravis
Summary Most primary immunodeficiency disorders ( PID ) are the result of single gene defects. Based on this fact, more than 240 different entities have been identified. Those PIDs with predominant antibody deficiency are treated with immunoglobulin ( I g) replacement therapy. This review focuses on the diagnosis, clinical characteristics and treatment of patients suffering from PID , or secondary immunodeficiency disorders ( SID ) caused, for instance, by irradiation, immunosuppressive drugs or thymectomy. Common variable immunodeficiency ( CVID ) is the most commonly diagnosed and least understood form of PID , with a heterogeneous range of symptoms and genotypes, requiring individualized treatment plans. This includes adjusting the dose and treatment interval, administrating I g by intravenous or subcutaneous injection by either pump or push, and finally deciding which treatment options are best for a given patient. Ig therapy can also be used to treat immunodeficiencies resulting from lymphoproliferative and autoimmune diseases or immunosuppression following organ transplantation; however, there is an urgent need for research in this field. Accurate and early diagnosis of PID is important to ensure that optimal treatment is started early to maintain the patient's health. Detailed patient registries have been established to increase awareness of PID , as well as provide a valuable resource for further research.