Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency.

Autor:	Pashangzadeh S; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Delavari S; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran.; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran., Moeini Shad T; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Salami F; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran.; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran., Rasouli SE; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran.; Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran., Yazdani R; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Mahdaviani SA; Pediatric Respiratory Disease Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti., Nabavi M; Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran., Aleyasin S; Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran., Ahanchian H; Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran., Jabbari-Azad F; Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran., Chavoshzadeh Z; Pediatric Infections Research Center, Mofid Children´s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran., Nazari F; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Momen T; Department of Allergy and Clinical Immunology, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Noncommunicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran., Sherkat R; Immunodeficiency Diseases Research Center, Isfahan University of Medical Sciences, Isfahan, Iran., Abolnezhadian F; Department of Pediatrics, Abuzar Children´s Hospital, Ahvaz University of Medical Sciences, Ahvaz, Iran., Esmaeilzadeh H; Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran., Fallahpour M; Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran., Arshi S; Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran., Bemanian MH; Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran., Shokri S; Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran., Ebrahimi SS; Department of Immunology and Allergy, Kerman University of Medical Sciences, Kerman, Iran., Abolmolouki M; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Farid AS; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Rezaei A; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Esmaeili M; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran.; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran., Kalantari A; Department of Immunology and Allergy, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran., Sadeghi-Shabestari M; Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran., Shirkani A; Allergy and Clinical Immunology Department, Bushehr University of Medical Sciences, School of Medicine, Bushehr, Iran., Behniafard N; Children Growth Disorder Research Center, Shahid Sadoughi University of Medical Sciences, Yazd, Iran., Khalili A; Department of Pediatrics, Shahid Sadoughi University of Medical Sciences, Yazd, Iran., Eslamian MH; Department of Pediatrics, Hamedan University of Medical Sciences, Hamedan, Iran., Cheraghi T; Department of Pediatrics, 17 Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran., Shafie A; Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran., Tavakol M; Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran., Khoshkhui M; Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran., Iranparast S; Department of Immunology, Faculty of Medicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran., Shamshiri M; Department of Immunology, Faculty of Medicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran., Shahri MA; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Khazaei R; Department of Pediatrics, Abuzar Children´s Hospital, Ahvaz University of Medical Sciences, Ahvaz, Iran., Asadi M; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Babaha F; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Aghamohammadi A; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran., Rezaei N; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran.; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran., Abolhassani H; Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran.; Division of Immunology, Department of Medical Biochemistry and Biophysics, Karolinska Institute, Stockholm, Sweden.
Jazyk:	angličtina
Zdroj:	Journal of investigational allergology & clinical immunology [J Investig Allergol Clin Immunol] 2024 Jul; Vol. 34 (4), pp. 233-245.
DOI:	10.18176/jiaci.0902
Abstrakt:	Background: Common variable immunodeficiency (CVID) is considered the most symptomatic type of inborn errors of immunity in humans. Along with infectious complications, which have numerous consequences, noninfectious complications are a major challenge among CVID patients. Methods: All CVID patients registered in the national database were included in this retrospective cohort study. Patients were divided into 2 groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, noninfectious organ involvement, autoimmunity, and lymphoproliferative diseases were evaluated. Results: Among 387 enrolled patients, 66.4% were diagnosed with noninfectious complications and 33.6% with isolated infectious presentations. Enteropathy, autoimmunity, and lymphoproliferative disorders were reported in 35.1%, 24.3%, and 21.4% of patients, respectively. Some complications, including autoimmunity and hepatosplenomegaly, were reported to be significantly more frequent among patients with B-cell lymphopenia. As for organ involvement, the dermatologic, endocrine, and musculoskeletal systems were predominantly affected in CVID patients with B-cell lymphopenia. Among autoimmune manifestations, the frequency of rheumatologic, hematologic, and gastrointestinal autoimmunity was reported to be higher than that of other types of autoimmunity not associated with B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were the most common type of malignancy. The mortality rate was 24.5%, and respiratory failure and malignancies were the most common causes of death, with no significant differences between the 2 groups. Conclusions: Considering that some of the noninfectious complications might be associated with B-cell lymphopenia, regular patient monitoring and follow-up with proper medication (in addition to immunoglobulin replacement therapy) are highly recommended to prevent sequelae and increase patient quality of life.
Databáze:	MEDLINE
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