Abstract
Common variable immunodeficiency (CVID) is the most clinically significant primary antibody immunodeficiency recognized in adulthood. Previously published data have shown an average diagnostic delay of 10 years for Polish adult patients with CVID. In the current study, we aimed to analyze the current diagnostic delay of adult patients with CVID in Poland. To this end, we identified patients from four immunological centers specialized in the care of adult patients with primary immunodeficiencies (PID). Demographic and clinical data of patients were collected using an internet database. We identified 103 adult patients (F:M 44.7%:55.3%) in Poland with CVID. The median age at onset of symptoms was 24 (0–66), 33 (4–70) at diagnosis, and 37 (18–73) years at the time of analysis. The median diagnostic delay for the entire study population was 6 (0–57) years. However, this delay was higher in patients with symptom onset before the year 2000 than after the year 1999 [15 (0–57) vs. 3 (0–19) years; p < 0.001]. Comparing patients (median ≤ 6 years, N = 53) with short diagnostic delay (SDD) and those (median > 6 years, N = 50) with long diagnostic delay (LDD), the LDD group had a statistically significant higher incidence of infections of the lower respiratory tract before diagnosis (90.0 vs. 71.70%). During the entire observation period, cytopenias (44.00 vs. 22.64%), granulomatous lesions (28.00 vs. 11.32%), and solid tumors (14.00 vs. 1.89%) were significantly more frequent in the LDD group. In conclusion, we found a significant reduction in the median diagnostic delay in Polish CVID patients with disease onset in the last two decades.
Highlights
This study aimed to determine the length of the diagnostic delay of common variable immunodeficiency (CVID) in a group of Polish adult patients and compare groups of patients with short (SDD) and long diagnostic delay (LDD)
Patients were treated at four immunological centers specializing in the care of adult patients with primary immunodeficiencies (Department of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Torun, Bydgoszcz; Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdansk, Gdansk; Outpatient Clinic for the Immunological and Hypercoagulable Diseases, The University Hospital in Krakow, Cracow; and Department of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw)
This study consisted of 103 adult patients, including 46 women (44.7%) and 57 men (55.3%) with CVID
Summary
Because of their innate nature, they are diagnosed mainly in childhood (1). More than half of PID cases are associated with a defect in antibody production or function (2). In this group, the most common symptomatic deficiency is common variable immunodeficiency (CVID) (1, 3). CVID is a heterogeneous group of disorders characterized by recurrent upper and lower respiratory tract infections, which occur in more than 85% of patients (4). Up to 70% of patients have at least one non-infectious manifestation, such as autoimmunization, granulomatous lesions, unexplained polyclonal lymphoproliferation, enteropathy, or malignancy (5–7)
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