Current clinical spectrum of common variable immunodeficiency in Spain: The multicentric nationwide GTEM-SEMI-CVID registry.
Autor: | Cabañero-Navalon MD; Department of Internal Medicine, University and Polytechnic Hospital LaFe, Valencia, Spain., Garcia-Bustos V; Department of Internal Medicine, University and Polytechnic Hospital LaFe, Valencia, Spain., Nuñez-Beltran M; Department of Internal Medicine, University and Polytechnic Hospital LaFe, Valencia, Spain., Císcar Fernández P; Department of Internal Medicine, University and Polytechnic Hospital LaFe, Valencia, Spain., Mateu L; Department of Internal Medicine, Germans Trias i Pujol University Hospital, Badalona, Spain., Solanich X; Department of Internal Medicine, Bellvitge University Hospital, Barcelona, Spain., Carrillo-Linares JL; Department of Internal Medicine, Virgen de la Victoria University Hospital, Málaga, Spain., Robles-Marhuenda Á; Department of Internal Medicine, La Paz University Hospital, Madrid, Madrid, Spain., Puchades-Gimeno F; Department of Internal Medicine, University General Hospital of Valencia, Valencia, Spain., Pelaez Ballesta A; Department of Internal Medicine, Rafael Méndez University Hospital, Murcia, Spain., López-Osle N; Department of Internal Medicine, Cruces University Hospital, Bizkaia, Spain., Torralba-Cabeza MÁ; Department of Internal Medicine, Lozano Blesa University Clinical Hospital, Zaragoza, Spain., Bielsa Masdeu AM; Department of Internal Medicine, Miguel Servet University Hospital, Zaragoza, Spain., Diego Gil J; Department of Internal Medicine, University Hospital October 12, Madrid, Spain., Tornador Gaya N; Department of Internal Medicine, University General Hospital of Castellón, Castellón, Spain., Pascual Castellanos G; Department of Internal Medicine, University General Hospital of Castellón, Castellón, Spain., Sánchez-Martínez R; Department of Internal Medicine, University General Hospital of Alicante, Alicante, Spain., Barragán-Casas JM; Department of Internal Medicine, Complejo Asistencial de Ávila, Ávila, Spain., González-García A; Department of Internal Medicine, Santiago Ramón y Cajal University Hospital, Madrid, Spain., Patier de la Peña JL; Department of Internal Medicine, Santiago Ramón y Cajal University Hospital, Madrid, Spain., López-Wolf D; Department of Internal Medicine, University Hospital Alcorcón Foundation, Madrid, Spain., Mora Rufete A; Department of Internal Medicine, General University Hospital of Elche, Alicante, Spain., Canovas Mora A; Department of Internal Medicine, General University Hospital of Elche, Alicante, Spain., Forner Giner MJ; Department of Internal Medicine, Clinical University Hospital of Valencia, Valencia, Spain., Moral Moral P; Department of Internal Medicine, University and Polytechnic Hospital LaFe, Valencia, Spain. |
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Jazyk: | angličtina |
Zdroj: | Frontiers in immunology [Front Immunol] 2022 Oct 28; Vol. 13, pp. 1033666. Date of Electronic Publication: 2022 Oct 28 (Print Publication: 2022). |
DOI: | 10.3389/fimmu.2022.1033666 |
Abstrakt: | Common variable immunodeficiency (CVID) constitutes a heterogenic group of primary immunodeficiency disorders with a wide-ranging clinical spectrum. CVID-associated non-infectious morbidity constitutes a major challenge requiring a full understanding of its pathophysiology and its clinical importance and global variability, especially considering the broad clinical, genetic, and regional heterogeneity of CVID disorders. This work aimed to develop a nationwide, multicenter, retrospective study over a 3-year period describing epidemiological, clinical, laboratory, therapeutic, and prognostic features of 250 CVID patients in Spain. The mean diagnostic delay was around 10 years and most patients initially presented with infectious complications followed by non-infectious immune disorders. However, infectious diseases were not the main cause of morbimortality. Non-infectious lung disease was extraordinarily frequent in our registry affecting approximately 60% of the patients. More than one-third of the patients in our cohort showed lymphadenopathies and splenomegaly in their follow-up, and more than 33% presented immune cytopenias, especially Evans' syndrome. Gastrointestinal disease was observed in more than 40% of the patients. Among biopsied organs in our cohort, benign lymphoproliferation was the principal histopathological alteration. Reaching 15.26%, the global prevalence of cancer in our registry was one of the highest reported to date, with non-Hodgkin B lymphoma being the most frequent. These data emphasize the importance of basic and translational research delving into the pathophysiological pathways involved in immune dysregulation and diffuse lymphocytic infiltration. This would reveal new tailored strategies to reduce immune complications, and the associated healthcare burden, and ensure a better quality of life for CVID patients. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2022 Cabañero-Navalon, Garcia-Bustos, Nuñez-Beltran, Císcar Fernández, Mateu, Solanich, Carrillo-Linares, Robles-Marhuenda, Puchades-Gimeno, Pelaez Ballesta, López-Osle, Torralba-Cabeza, Bielsa Masdeu, Diego Gil, Tornador Gaya, Pascual Castellanos, Sánchez-Martínez, Barragán-Casas, González-García, Patier de la Peña, López-Wolf, Mora Rufete, Canovas Mora, Forner Giner and Moral Moral.) |
Databáze: | MEDLINE |
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