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Interstitial Lung Disease in Common Variable Immunodeficiency

Interstitial lung disease (ILD) is a common complication in patients with common variable immunodeficiency (CVID) and often associated with other features, such as bronchiectasis and autoimmunity. As the ILD term encompasses different acute and chronic pulmonary conditions, the diagnosis is commonly...

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Autores principales: Lopes, Joao Pedro, Ho, Hsi-en, Cunningham-Rundles, Charlotte
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7990881/
https://www.ncbi.nlm.nih.gov/pubmed/33776995
http://dx.doi.org/10.3389/fimmu.2021.605945
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author Lopes, Joao Pedro
Ho, Hsi-en
Cunningham-Rundles, Charlotte
author_facet Lopes, Joao Pedro
Ho, Hsi-en
Cunningham-Rundles, Charlotte
author_sort Lopes, Joao Pedro
collection PubMed
description Interstitial lung disease (ILD) is a common complication in patients with common variable immunodeficiency (CVID) and often associated with other features, such as bronchiectasis and autoimmunity. As the ILD term encompasses different acute and chronic pulmonary conditions, the diagnosis is commonly made based on imaging features; histopathology is less frequently available. From a cohort of 637 patients with CVID followed at our center over 4 decades, we reviewed the data for 46 subjects (30 females, 16 males) who had lung biopsies with proven ILD. They had a median age at CVID diagnosis of 26 years old, with a median IgG level at diagnosis of 285.0 mg/dL with average isotype switched memory B cells of 0.5%. Lung biopsy pathology revealed granulomas in 25 patients (54.4%), lymphoid interstitial pneumonia in 13 patients (28.3%), lymphoid hyperplasia not otherwise specified in 7 patients (15.2%), cryptogenic organizing pneumonia in 7 patients (15.2%), follicular bronchitis in 4 patients (8.7%), and predominance of pulmonary fibrosis in 4 patients (8.7%). Autoimmune manifestations were common and were present in 28 (60.9%) patients. Nine patients (19.6%) died, with a median age at death of 49-years-old. Lung transplant was done in 3 of these patients (6.5%) who are no longer alive. These analyses reveal the high burden of this complication, with almost one-fifth of the group deceased in this period. Further understanding of the causes of the development and progression of ILD in CVID patients is required to define the best management for this patient population.
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spelling pubmed-79908812021-03-26 Interstitial Lung Disease in Common Variable Immunodeficiency Lopes, Joao Pedro Ho, Hsi-en Cunningham-Rundles, Charlotte Front Immunol Immunology Interstitial lung disease (ILD) is a common complication in patients with common variable immunodeficiency (CVID) and often associated with other features, such as bronchiectasis and autoimmunity. As the ILD term encompasses different acute and chronic pulmonary conditions, the diagnosis is commonly made based on imaging features; histopathology is less frequently available. From a cohort of 637 patients with CVID followed at our center over 4 decades, we reviewed the data for 46 subjects (30 females, 16 males) who had lung biopsies with proven ILD. They had a median age at CVID diagnosis of 26 years old, with a median IgG level at diagnosis of 285.0 mg/dL with average isotype switched memory B cells of 0.5%. Lung biopsy pathology revealed granulomas in 25 patients (54.4%), lymphoid interstitial pneumonia in 13 patients (28.3%), lymphoid hyperplasia not otherwise specified in 7 patients (15.2%), cryptogenic organizing pneumonia in 7 patients (15.2%), follicular bronchitis in 4 patients (8.7%), and predominance of pulmonary fibrosis in 4 patients (8.7%). Autoimmune manifestations were common and were present in 28 (60.9%) patients. Nine patients (19.6%) died, with a median age at death of 49-years-old. Lung transplant was done in 3 of these patients (6.5%) who are no longer alive. These analyses reveal the high burden of this complication, with almost one-fifth of the group deceased in this period. Further understanding of the causes of the development and progression of ILD in CVID patients is required to define the best management for this patient population. Frontiers Media S.A. 2021-03-11 /pmc/articles/PMC7990881/ /pubmed/33776995 http://dx.doi.org/10.3389/fimmu.2021.605945 Text en Copyright © 2021 Lopes, Ho and Cunningham-Rundles. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Lopes, Joao Pedro
Ho, Hsi-en
Cunningham-Rundles, Charlotte
Interstitial Lung Disease in Common Variable Immunodeficiency
title Interstitial Lung Disease in Common Variable Immunodeficiency
title_full Interstitial Lung Disease in Common Variable Immunodeficiency
title_fullStr Interstitial Lung Disease in Common Variable Immunodeficiency
title_full_unstemmed Interstitial Lung Disease in Common Variable Immunodeficiency
title_short Interstitial Lung Disease in Common Variable Immunodeficiency
title_sort interstitial lung disease in common variable immunodeficiency
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7990881/
https://www.ncbi.nlm.nih.gov/pubmed/33776995
http://dx.doi.org/10.3389/fimmu.2021.605945
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