Lung disease, T-cells and inflammation in common variable immunodeficiency disorders

Gregersen, Stina; Holm, Are Martin; Fevang, Børre; Ueland, Thor; Sikkeland, Liv Ingunn Bjoner; Aaløkken, Trond Mogens; Mynarek, Georg; Kongerud, Johny; Aukrust, Pål; Johansen, B.V.; Frøland, Stig S.

doi:10.3109/00365513.2013.819523

Cited by 14 publications

(9 citation statements)

References 40 publications

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“…Splenomegaly can be a biomarker for GLILD, given the multi-systemic nature of the disease ( 81 ). Abnormalities in the T-cell population found in BALF, such as a low CD4/CD8 ratio, can be associated with poor functional outcomes including FEV1 and FVC ( 137 ). However, bronchoalveolar lavage for T-cell assessment is an invasive technique and thus it is usually used only in patients with severe disease.…”

Section: Assessment Of the Lung In Padmentioning

confidence: 99%

The Lung in Primary Immunodeficiencies: New Concepts in Infection and Inflammation

et al. 2018

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Immunoglobulin replacement therapy (IGRT) has contributed critically to the management of primary antibody deficiencies (PAD) and the decrease in pneumonia rate. However, despite adequate IGRT and improved prognosis, patients with PAD continue to experience recurrent respiratory tract infections, leading to bronchiectasis and continuing decline in lung function with a severe impact on their quality of life. Moreover, non-infectious inflammatory and interstitial lung complications, such as granulomatous-lymphocytic interstitial lung disease, contribute substantially to the overall morbidity of PAD. These conditions develop much more often than appreciated and represent a major therapeutic challenge. Therefore, a regular assessment of the structural and functional condition of the lung and the upper airways with appropriate treatment is required to minimize the deterioration of lung function. This work summarizes the knowledge on lung complications in PAD and discusses the currently available diagnostic tools and treatment options.

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Section: Assessment Of the Lung In Padmentioning

confidence: 99%

The Lung in Primary Immunodeficiencies: New Concepts in Infection and Inflammation

et al. 2018

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“…The CD4/CD8 preponderance has varied between studies, and a higher ratio has been associated with a more favourable prognosis in terms of lung function decline in one study [53] , but not in another [54] . It is recognised that abnormalities present in blood lymphocyte populations in CVID may also be present in BAL [55] , but in general there is a paucity of data on lung lymphocyte populations in CVID One study [53] included an assessment with trans-bronchial biopsy but this was not adequate to make a firm diagnosis in over 2/3 of patients.…”

Section: Figure 5: Histology Of Glild Demonstrating a Polymorphous Inmentioning

confidence: 99%

Lung disease in primary antibody deficiency

Verma

Grimbacher

Hurst

2015

The Lancet Respiratory Medicine

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This Review summarises current knowledge on the pulmonary manifestations of primary antibody deficiency (PAD) syndromes in adults. We describe the major PAD syndromes, with a particular focus on common variable immunodeficiency (CVID). Respiratory infection is a common presenting feature of PAD syndromes. Respiratory complications are frequent and responsible for much of the morbidity and mortality associated with these syndromes. Respiratory complications include acute infections, the sequelae of infection (eg, bronchiectasis), non-infectious immune-mediated manifestations (notably the development of granulomatous-lymphocytic interstitial lung disease in CVID), and an increased risk of lymphoma. Although minor abnormalities are detectable in the lungs of most patients with CVID by CT scanning, not all patients develop lung complications. Mechanisms associated with the maintenance of lung health versus lung disease, and the development of bronchiectasis versus immune-mediated complications, are now being dissected. We review the investigation, treatment, and management strategies for PAD syndromes, and include key research questions relating to both infectious and non-infectious complications of PAD in the lung.

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“…Although as many as 50% of CVID patients reportedly develop bronchiectasis, not all of whom have or progress to ILD. 11 Development of lung disease in CVID patients has previously been associated with a low CD4+:CD8+ T cell ratio in bronchoalveolar lavage 22 as well as reductions in peripheral CD8+ T cells 6 and fewer numbers of IgM-IgD-CD27+ isotype-switched as well as IgM+CD27+ memory B cells, 23,24 in some, but not all studies. 25 Epstein-Barr Virus may be associated with PLH, 26 including subjects with HIV, 27 however EBV has not been found in lung biopsies from CVID patients with PLH.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary radiologic findings in common variable immunodeficiency: clinical and immunological correlations

Maglione

Overbey

Radigan

et al. 2014

Annals of Allergy, Asthma & Immunology

104

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Lung disease, T-cells and inflammation in common variable immunodeficiency disorders

Abstract: Our findings suggest that inflammation and T-cell activation may be involved in the immunopathogenesis of pulmonary complications in CVID.

Cited by 14 publications

References 40 publications

The Lung in Primary Immunodeficiencies: New Concepts in Infection and Inflammation

The Lung in Primary Immunodeficiencies: New Concepts in Infection and Inflammation

Lung disease in primary antibody deficiency

Pulmonary radiologic findings in common variable immunodeficiency: clinical and immunological correlations

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