期刊
ENDOCRINE METABOLIC & IMMUNE DISORDERS-DRUG TARGETS
卷 20, 期 2, 页码 157-171出版社
BENTHAM SCIENCE PUBL LTD
DOI: 10.2174/1871530319666190828125316
关键词
Primary immunodeficiency disorders; infection; antibody deficiency; combined immunodeficiency; autoimmunity; enteropathy
资金
- Alborz University of Medical Sciences [1397-03-25-1792]
Background and Objective: Primary immunodeficiency diseases (PIDs) are a group of more than 350 disorders affecting distinct components of the innate and adaptive immune systems. In this review, the classic and advanced stepwise approach towards the diagnosis of PIDs are simplified and explained in detail. Results: Susceptibility to recurrent infections is the main hallmark of almost all PIDs. However, non-infectious complications attributable to immune dysregulation presenting with lymphoproliferative and/or autoimmune disorders are not uncommon. Moreover, PIDs could be associated with misleading presentations including allergic manifestations, enteropathies, and malignancies. Conclusion: Timely diagnosis is the most essential element in improving outcome and reducing the morbidity and mortality in PIDs. This wouldn't be possible unless the physicians keep the diagnosis of PID in mind and be sufficiently aware of the approach to these patients.
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