Humoral Immune Status in Relation to Outcomes in Patients with Idiopathic Pulmonary Fibrosis.

Autor: Hoffman TW; Department of Pulmonology, St. Antonius Hospital, Koekoekslaan 1, 3435CM, Nieuwegein, The Netherlands. t.hoffman@antoniusziekenhuis.nl., van Moorsel CHM; Department of Pulmonology, St. Antonius Hospital, Koekoekslaan 1, 3435CM, Nieuwegein, The Netherlands.; Division of Heart and Lungs, University Medical Centre, Utrecht, The Netherlands., Kazemier KM; Department of Pulmonology, St. Antonius Hospital, Koekoekslaan 1, 3435CM, Nieuwegein, The Netherlands.; Division of Heart and Lungs, University Medical Centre, Utrecht, The Netherlands.; Centre for Translational Immunology, University Medical Centre, Utrecht, The Netherlands., Biesma DH; Department of Internal Medicine, St. Antonius Hospital, Nieuwegein, The Netherlands., Grutters JC; Department of Pulmonology, St. Antonius Hospital, Koekoekslaan 1, 3435CM, Nieuwegein, The Netherlands.; Division of Heart and Lungs, University Medical Centre, Utrecht, The Netherlands., van Kessel DA; Department of Pulmonology, St. Antonius Hospital, Koekoekslaan 1, 3435CM, Nieuwegein, The Netherlands.; Division of Heart and Lungs, University Medical Centre, Utrecht, The Netherlands.
Jazyk: angličtina
Zdroj: Lung [Lung] 2021 Dec; Vol. 199 (6), pp. 667-676. Date of Electronic Publication: 2021 Oct 29.
DOI: 10.1007/s00408-021-00488-w
Abstrakt: Purpose: Idiopathic pulmonary fibrosis (IPF) is a severe fibrotic lung disease, in which inflammation is thought to only play a secondary role. Several factors associated with acute exacerbations of IPF (AE-IPF) have been identified, including infections. This study investigated whether humoral immunodeficiency or increased inflammatory markers at diagnosis were associated with AE-IPF and survival.
Methods: Four-hundred-and-nine patients diagnosed with IPF between 2011 and 2017 were retrospectively included. Immune status investigations at diagnosis included measurement of serum immunoglobulins (available in 38%), leukocyte and lymphocyte subsets in blood and bronchoalveolar lavage (BAL) fluid (available in 58%), as well as response to pneumococcal vaccination (available in 64%).
Results: Serum immunoglobulins or IgG subclass levels were below the lower limit of normal in 6%. The response to pneumococcal vaccination was severely impaired in 1%. Thirteen percent of patients developed an AE-IPF (4.7% per year). AE-IPF were associated with elevated lymphocytes in BAL fluid at diagnosis (p = 0.03). Higher serum IgA and IgG at diagnosis were associated with worse survival (p = 0.01; and p = 0.04), as were an increased BAL lymphocyte percentage (p = 0.005), and higher blood leukocytes and neutrophils (p = 0.01; and p = 0.0005). In a multivariate model, only BAL lymphocyte count retained statistical significance (p = 0.007).
Conclusion: The prevalence of humoral immunodeficiencies was low in patients with IPF and not associated with AE-IPF or survival. Elevated lymphocytes in BAL were associated with the development of AE-IPF and worse survival. Higher serum immunoglobulins and immune cells in blood were also associated with worse survival. The local immune response in the lungs may be a target for future therapies.
(© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
Databáze: MEDLINE
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