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Allergy and asthma proceedings · 2025

Comparative analysis of demographic and clinical features in common variable immunodeficiency and selective immunoglobulin G deficiency.

СтатьяСравнительное исследованиеНазвание и аннотация: Перевод в очереди
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Gerek Mehmet Emin, Colkesen Fatih, Onalan Tugba, Akkus Fatma Arzu, Kilinc Mehmet, Evcen Recep, Kahraman Selim, Arslan Sevket

Аннотация

Background: Common variable immunodeficiency (CVID) and selective immunoglobulin G (IgG) deficiency (sIgGD) are primary antibody deficiencies with divergent clinical trajectories. CVID is characterized by recurrent infections and systemic noninfectious complications, whereas sIgGD typically follows a milder course. Despite sharing clinical features such as recurrent infections, comparative data on complications and mortality remain limited, which hinders risk-stratified management. Objective: This study compared the prevalence, clinical impact, and mortality associated with noninfectious complications in CVID and sIgGD, and identified predictors of adverse outcomes to support risk-stratified management and examined clinical differences within the CVID group based on baseline serum IgM levels. Methods: A single-center retrospective cohort study (2018-2024) included 111 patients with CVID and 19 patients with sIgGD. Diagnostic criteria for CVID included hypogammaglobulinemia (IgG level < 400 mg/dL with low IgA/IgM), impaired vaccine responses, and exclusion of secondary causes. The sIgGD required isolated IgG deficiency with normal IgA/IgM and intact vaccine responses. Noninfectious complications, including organ-specific and systemic manifestations as well as bronchiectasis were evaluated. In patients with CVID, a predefined subgroup analysis was performed based on baseline serum IgM levels (normal versus <40 mg/dL). Multivariate logistic regression identified mortality predictors. Results: The patients with CVID exhibited significantly higher rates of recurrent sinopulmonary infections (70.3% versus 42.1%), bronchiectasis (46.8% versus 21.1%), and noninfectious complications (57.7% versus 31.6%), including autoimmune disorders such as hematologic manifestations (33.3% versus 10.5%). In the CVID group, the patients with low IgM levels exhibited a significantly higher prevalence of gastrointestinal involvement (24.0% versus 5.6%; p = 0.035). Bronchiectasis (odds ratio [OR] 5.22) and noninfectious complications (OR 7.95) independently predicted mortality in CVID. Male sex showed borderline risk. In contrast, no mortality was observed in the sIgGD cohort over the study period. Conclusion: CVID is associated with substantial noninfectious morbidity and mortality, which necessitates early identification and long-term monitoring. In contrast, sIgGD exhibits a milder clinical phenotype, although preventive care remains important. These findings support distinct management strategies and highlight the utility of clinical markers for risk stratification.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты