Primary Immune Regulatory Disorders and Targeted Therapies
Primary immune regulatory disorders (PIRDs) are a group of diseases belonging to inborn errors of immunity. They usually exhibit lymphoproliferation, autoimmunities, and malignancies, with less susceptibility to recurrent infections. Unlike classical primary immune deficiencies, in autoimmune manife...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Galenos Publishing House
2021-02-01
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| Series: | Turkish Journal of Hematology |
| Subjects: | |
| Online Access: | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tjh&un=TJH-90094 |
| _version_ | 1797920997211373568 |
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| author | Burcu Kolukısa Safa Barış |
| author_facet | Burcu Kolukısa Safa Barış |
| author_sort | Burcu Kolukısa |
| collection | DOAJ |
| description | Primary immune regulatory disorders (PIRDs) are a group of diseases belonging to inborn errors of immunity. They usually exhibit lymphoproliferation, autoimmunities, and malignancies, with less susceptibility to recurrent infections. Unlike classical primary immune deficiencies, in autoimmune manifestations, such as cytopenias, enteropathy can be the first symptom of diseases, and they are typically resistant to treatment. Increasing awareness of PIRDs among specialists and a multidisciplinary team approach would provide early diagnosis and treatment that could prevent end-organ damage related to the diseases. In recent years, many PIRDs have been described, and understanding the immunological pathways linked to these disorders provides us an opportunity to use directed therapies for specific molecules, which usually offer better disease control than known classical immunosuppressants. In this review, in light of the most recent literature, we will discuss the common PIRDs and explain their clinical symptoms and recent treatment modalities. |
| first_indexed | 2024-04-10T14:09:42Z |
| format | Article |
| id | doaj.art-3b6a989671f6429cbc4135bc6c96bd7e |
| institution | Directory Open Access Journal |
| issn | 1308-5263 |
| language | English |
| last_indexed | 2024-04-10T14:09:42Z |
| publishDate | 2021-02-01 |
| publisher | Galenos Publishing House |
| record_format | Article |
| series | Turkish Journal of Hematology |
| spelling | doaj.art-3b6a989671f6429cbc4135bc6c96bd7e2023-02-15T16:09:47ZengGalenos Publishing HouseTurkish Journal of Hematology1308-52632021-02-0138111410.4274/tjh.galenos.2021.2020.0724TJH-90094Primary Immune Regulatory Disorders and Targeted TherapiesBurcu Kolukısa0Safa Barış1Marmara University Faculty of Medicine, Division of Pediatric Allergy and Immunology, İstanbul, Turkey; İstanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, İstanbul, Turkey; The Işıl Berat Barlan Center for Translational Medicine, İstanbul, TurkeyMarmara University Faculty of Medicine, Division of Pediatric Allergy and Immunology, İstanbul, Turkey; İstanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies, İstanbul, Turkey; The Işıl Berat Barlan Center for Translational Medicine, İstanbul, TurkeyPrimary immune regulatory disorders (PIRDs) are a group of diseases belonging to inborn errors of immunity. They usually exhibit lymphoproliferation, autoimmunities, and malignancies, with less susceptibility to recurrent infections. Unlike classical primary immune deficiencies, in autoimmune manifestations, such as cytopenias, enteropathy can be the first symptom of diseases, and they are typically resistant to treatment. Increasing awareness of PIRDs among specialists and a multidisciplinary team approach would provide early diagnosis and treatment that could prevent end-organ damage related to the diseases. In recent years, many PIRDs have been described, and understanding the immunological pathways linked to these disorders provides us an opportunity to use directed therapies for specific molecules, which usually offer better disease control than known classical immunosuppressants. In this review, in light of the most recent literature, we will discuss the common PIRDs and explain their clinical symptoms and recent treatment modalities.https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tjh&un=TJH-90094primary immune deficienciesimmune dysregulationautoimmunitylymphoproliferationprecision medicinetargeted therapy |
| spellingShingle | Burcu Kolukısa Safa Barış Primary Immune Regulatory Disorders and Targeted Therapies Turkish Journal of Hematology primary immune deficiencies immune dysregulation autoimmunity lymphoproliferation precision medicine targeted therapy |
| title | Primary Immune Regulatory Disorders and Targeted Therapies |
| title_full | Primary Immune Regulatory Disorders and Targeted Therapies |
| title_fullStr | Primary Immune Regulatory Disorders and Targeted Therapies |
| title_full_unstemmed | Primary Immune Regulatory Disorders and Targeted Therapies |
| title_short | Primary Immune Regulatory Disorders and Targeted Therapies |
| title_sort | primary immune regulatory disorders and targeted therapies |
| topic | primary immune deficiencies immune dysregulation autoimmunity lymphoproliferation precision medicine targeted therapy |
| url | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tjh&un=TJH-90094 |
| work_keys_str_mv | AT burcukolukısa primaryimmuneregulatorydisordersandtargetedtherapies AT safabarıs primaryimmuneregulatorydisordersandtargetedtherapies |