Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999
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 curren...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2020-05-01
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| Series: | Frontiers in Immunology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fimmu.2020.00982/full |
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| author | Marcin Ziętkiewicz Ewa Więsik-Szewczyk Aleksandra Matyja-Bednarczyk Katarzyna Napiórkowska-Baran Zbigniew Zdrojewski Karina Jahnz-Różyk |
| author_facet | Marcin Ziętkiewicz Ewa Więsik-Szewczyk Aleksandra Matyja-Bednarczyk Katarzyna Napiórkowska-Baran Zbigniew Zdrojewski Karina Jahnz-Różyk |
| author_sort | Marcin Ziętkiewicz |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-11T05:56:11Z |
| format | Article |
| id | doaj.art-4fac2414864c4fff83b0078d6cc15c69 |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-12-11T05:56:11Z |
| publishDate | 2020-05-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-4fac2414864c4fff83b0078d6cc15c692022-12-22T01:18:39ZengFrontiers Media S.A.Frontiers in Immunology1664-32242020-05-011110.3389/fimmu.2020.00982539581Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999Marcin Ziętkiewicz0Ewa Więsik-Szewczyk1Aleksandra Matyja-Bednarczyk2Katarzyna Napiórkowska-Baran3Zbigniew Zdrojewski4Karina Jahnz-Różyk5Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdansk, Gdansk, PolandDepartment of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, PolandOutpatient Clinic for the Immunological and Hypercoagulable Diseases, The University Hospital in Krakow, Kraków, PolandDepartment of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Torun, Bydgoszcz, PolandDepartment of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdansk, Gdansk, PolandDepartment of Internal Medicine, Pneumonology, Allergology and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, PolandCommon 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.https://www.frontiersin.org/article/10.3389/fimmu.2020.00982/fullprimary antibody deficiencyhypogammaglobulinemiacommon variable immunodeficiencydiagnostic delayadultsepidemiology |
| spellingShingle | Marcin Ziętkiewicz Ewa Więsik-Szewczyk Aleksandra Matyja-Bednarczyk Katarzyna Napiórkowska-Baran Zbigniew Zdrojewski Karina Jahnz-Różyk Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999 Frontiers in Immunology primary antibody deficiency hypogammaglobulinemia common variable immunodeficiency diagnostic delay adults epidemiology |
| title | Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999 |
| title_full | Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999 |
| title_fullStr | Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999 |
| title_full_unstemmed | Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999 |
| title_short | Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999 |
| title_sort | shorter diagnostic delay in polish adult patients with common variable immunodeficiency and symptom onset after 1999 |
| topic | primary antibody deficiency hypogammaglobulinemia common variable immunodeficiency diagnostic delay adults epidemiology |
| url | https://www.frontiersin.org/article/10.3389/fimmu.2020.00982/full |
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