Pulmonary Complications of Monogenic Patients with Common Variable Immunodeficiency: COVID-19 Perspectives
Pulmonary complications are one the main causes of morbidity and mortality in patients with common variable immunodeficiency (CVID). Although CVID pathogenesis is not completely understood, several genes have been identified to mainly regulate the process of terminal B-cell differentiation, antibody...
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| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
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Galenos Publishing House
2024-04-01
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| Series: | Turkish Journal of Immunology |
| Subjects: | |
| Online Access: | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tji&un=TJI-08860 |
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| author | Samaneh Delavari Marzie Esmaeili Fereshte Salami Seyed Erfan Rasouli Saba Fekrvand Mahsa Yousefpour Marzbali Nazanin Fathi Hassan Abolhassani |
| author_facet | Samaneh Delavari Marzie Esmaeili Fereshte Salami Seyed Erfan Rasouli Saba Fekrvand Mahsa Yousefpour Marzbali Nazanin Fathi Hassan Abolhassani |
| author_sort | Samaneh Delavari |
| collection | DOAJ |
| description | Pulmonary complications are one the main causes of morbidity and mortality in patients with common variable immunodeficiency (CVID). Although CVID pathogenesis is not completely understood, several genes have been identified to mainly regulate the process of terminal B-cell differentiation, antibody isotype maturation and long-life memory/plasma cell generation. The link between underlying genetic defects and the prognosis of developing different clinical complications like different pulmonary manifestations is still elusive. We provide an overview of recent advancements in the monogenic form of CVID which lead to dysregulation of B-cells at different levels of cytokine stimulations, intracellular signaling, transcription-factor activation, gene transcription and epigenetic controls and predispose patients to different pulmonary complications. The susceptibility to Coronavirus disease 2019 pulmonary complications was discussed in these patients. Monogenic forms of CVID have a distinct pattern in different infectious and non-infectious pulmonary manifestations including autoimmunity, atopy, lymphoproliferation, and cancer. |
| format | Article |
| id | doaj-art-b1974014994341699e821fa9e1938789 |
| institution | Kabale University |
| issn | 1301-109X 2147-8325 |
| language | English |
| publishDate | 2024-04-01 |
| publisher | Galenos Publishing House |
| record_format | Article |
| series | Turkish Journal of Immunology |
| spelling | doaj-art-b1974014994341699e821fa9e19387892025-01-17T13:55:52ZengGalenos Publishing HouseTurkish Journal of Immunology1301-109X2147-83252024-04-0112Suppl 1718210.4274/tji.galenos.2023.08860TJI-08860Pulmonary Complications of Monogenic Patients with Common Variable Immunodeficiency: COVID-19 PerspectivesSamaneh Delavari0Marzie Esmaeili1Fereshte Salami2Seyed Erfan Rasouli3Saba Fekrvand4Mahsa Yousefpour Marzbali5Nazanin Fathi6Hassan Abolhassani7Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, IranKarolinska Institutet, Department of Medical Biochemistry and Biophysics, Division of Immunology, Stockholm, SwedenPulmonary complications are one the main causes of morbidity and mortality in patients with common variable immunodeficiency (CVID). Although CVID pathogenesis is not completely understood, several genes have been identified to mainly regulate the process of terminal B-cell differentiation, antibody isotype maturation and long-life memory/plasma cell generation. The link between underlying genetic defects and the prognosis of developing different clinical complications like different pulmonary manifestations is still elusive. We provide an overview of recent advancements in the monogenic form of CVID which lead to dysregulation of B-cells at different levels of cytokine stimulations, intracellular signaling, transcription-factor activation, gene transcription and epigenetic controls and predispose patients to different pulmonary complications. The susceptibility to Coronavirus disease 2019 pulmonary complications was discussed in these patients. Monogenic forms of CVID have a distinct pattern in different infectious and non-infectious pulmonary manifestations including autoimmunity, atopy, lymphoproliferation, and cancer.https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tji&un=TJI-08860primary immunodeficiencyinborn errors of immunitycommon variable immunodeficiencygeneticrespiratory complications |
| spellingShingle | Samaneh Delavari Marzie Esmaeili Fereshte Salami Seyed Erfan Rasouli Saba Fekrvand Mahsa Yousefpour Marzbali Nazanin Fathi Hassan Abolhassani Pulmonary Complications of Monogenic Patients with Common Variable Immunodeficiency: COVID-19 Perspectives Turkish Journal of Immunology primary immunodeficiency inborn errors of immunity common variable immunodeficiency genetic respiratory complications |
| title | Pulmonary Complications of Monogenic Patients with Common Variable Immunodeficiency: COVID-19 Perspectives |
| title_full | Pulmonary Complications of Monogenic Patients with Common Variable Immunodeficiency: COVID-19 Perspectives |
| title_fullStr | Pulmonary Complications of Monogenic Patients with Common Variable Immunodeficiency: COVID-19 Perspectives |
| title_full_unstemmed | Pulmonary Complications of Monogenic Patients with Common Variable Immunodeficiency: COVID-19 Perspectives |
| title_short | Pulmonary Complications of Monogenic Patients with Common Variable Immunodeficiency: COVID-19 Perspectives |
| title_sort | pulmonary complications of monogenic patients with common variable immunodeficiency covid 19 perspectives |
| topic | primary immunodeficiency inborn errors of immunity common variable immunodeficiency genetic respiratory complications |
| url | https://jag.journalagent.com/z4/download_fulltext.asp?pdir=tji&un=TJI-08860 |
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