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Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency
S Pashangzadeh1, S Delavari1,2, T Moeini Shad1
1Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran.
Insights
Common variable immunodeficiency (CVID) patients frequently experience noninfectious complications, especially those with B-cell lymphopenia. Early monitoring and treatment are crucial for managing CVID complications and improving patient outcomes.
Area of Science:
- Immunology
- Hematology
- Genetics
Background:
- Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by significant symptomatic manifestations.
- Noninfectious complications pose a major challenge for CVID patients, alongside frequent infectious issues.
Purpose of the Study:
- To investigate the prevalence and characteristics of noninfectious complications in CVID patients.
- To determine the association between B-cell lymphopenia and specific noninfectious manifestations in CVID.
Main Methods:
- Retrospective cohort study of CVID patients from a national database.
- Patients categorized into groups based on the presence or absence of B-cell lymphopenia.
- Evaluation of demographic data, laboratory results, organ involvement, autoimmunity, and lymphoproliferative diseases.
Main Results:
- Noninfectious complications were diagnosed in 66.4% of 387 CVID patients.
- Enteropathy, autoimmunity, and lymphoproliferative disorders were common, affecting 35.1%, 24.3%, and 21.4% respectively.
- B-cell lymphopenia was associated with increased frequency of autoimmunity, specific organ involvement (dermatologic, endocrine, musculoskeletal), and certain autoimmune manifestations (rheumatologic, hematologic, gastrointestinal).
Conclusions:
- Noninfectious complications, particularly those linked to B-cell lymphopenia, necessitate regular patient monitoring.
- Comprehensive management including medication beyond immunoglobulin replacement therapy is recommended.
- Proactive follow-up aims to prevent sequelae and enhance the quality of life for CVID patients.
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.
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