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  3. Current clinical spectrum of common variable immunodeficiency in Spain: The multicentric nationwide GTEM-SEMI-CVID registry

Current clinical spectrum of common variable immunodeficiency in Spain: The multicentric nationwide GTEM-SEMI-CVID registry

Fecha de publicación:

Autores de IIS La Fe

Participantes ajenos a IIS La Fe

  • Cabañero-Navalon M.D.
  • Mateu L.
  • Solanich X.
  • Carrillo-Linares J.L.
  • Robles-Marhuenda Á.
  • Puchades-Gimeno F.
  • Pelaez Ballesta A.
  • López-Osle N.
  • Torralba-Cabeza M.Á.
  • Bielsa Masdeu A.M.
  • Diego Gil J.
  • Tornador Gaya N.
  • Pascual Castellanos G.
  • Sánchez-Martínez R.
  • Barragán-Casas J.M.
  • González-García A.
  • Patier de la Peña J.L.
  • López-Wolf D.
  • Mora Rufete A.
  • Canovas Mora A.
  • Forner Giner M.J.

Grupos

Abstract

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. 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.

Datos de la publicación

ISSN/ISSNe:
1664-3224, 1664-3224

Frontiers in Immunology  FRONTIERS MEDIA SA

Tipo:
Article
Páginas:
-
PubMed:
36389743
Enlace a otro recurso:
www.scopus.com
Factor de Impacto:
2,331 SCImago ℠
Cuartil:
Q1 SCImago ℠

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Keywords

  • Common Variable Immunodeficiency; Delayed Diagnosis; Humans; Lymphoma, Non-Hodgkin; Quality of Life; Registries; Retrospective Studies; Spain; azathioprine; high density lipoprotein; low density lipoprotein; rheumatoid factor; rituximab; triacylglycerol; abdominal pain; adult; Article; bronchiolitis; CD4 lymphocyte count; cell heterogeneity; common variable immunodeficiency; Crohn disease; cross-sectional study; cryptococcosis; cytomegalovirus infection; cytopenia; dyspnea; Evans syndrome; female; granulomatosis; Helicobacter pylori; hepatomegaly; histopathology; Hodgkin disease; human; immune dysregulation; interstitial pneumonia; leishmaniasis; lung biopsy; lymphadenopathy; lymphocyte proliferation; lymphocytic colitis; lymphocytic infiltration; major clinical study; male; middle aged; morbidity; multicenter study; natural killer cell; nodular hyperplasia; pathophysiology; Pneumocystis pneumonia; portal hypertension; splenomegaly; tuberculosis; clinical trial; common variable immunod

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