Paradoxical Immune Responses in Non-HIV Cryptococcal Meningitis.

The fungus Cryptococcus is a major cause of meningoencephalitis in HIV-infected as well as HIV-uninfected individuals with mortalities in developed countries of 20% and 30%, respectively. In HIV-related disease, defects in T-cell immunity are paramount, whereas there is little understanding of mecha...

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Main Authors: Anil A Panackal, Simone C Wuest, Yen-Chih Lin, Tianxia Wu, Nannan Zhang, Peter Kosa, Mika Komori, Andrew Blake, Sarah K Browne, Lindsey B Rosen, Ferry Hagen, Jacques Meis, Stuart M Levitz, Martha Quezado, Dima Hammoud, John E Bennett, Bibi Bielekova, Peter R Williamson
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2015-05-01
Series:PLoS Pathogens
Online Access:https://doi.org/10.1371/journal.ppat.1004884
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author Anil A Panackal
Simone C Wuest
Yen-Chih Lin
Tianxia Wu
Nannan Zhang
Peter Kosa
Mika Komori
Andrew Blake
Sarah K Browne
Lindsey B Rosen
Ferry Hagen
Jacques Meis
Stuart M Levitz
Martha Quezado
Dima Hammoud
John E Bennett
Bibi Bielekova
Peter R Williamson
author_facet Anil A Panackal
Simone C Wuest
Yen-Chih Lin
Tianxia Wu
Nannan Zhang
Peter Kosa
Mika Komori
Andrew Blake
Sarah K Browne
Lindsey B Rosen
Ferry Hagen
Jacques Meis
Stuart M Levitz
Martha Quezado
Dima Hammoud
John E Bennett
Bibi Bielekova
Peter R Williamson
author_sort Anil A Panackal
collection DOAJ
description The fungus Cryptococcus is a major cause of meningoencephalitis in HIV-infected as well as HIV-uninfected individuals with mortalities in developed countries of 20% and 30%, respectively. In HIV-related disease, defects in T-cell immunity are paramount, whereas there is little understanding of mechanisms of susceptibility in non-HIV related disease, especially that occurring in previously healthy adults. The present description is the first detailed immunological study of non-HIV-infected patients including those with severe central nervous system (s-CNS) disease to 1) identify mechanisms of susceptibility as well as 2) understand mechanisms underlying severe disease. Despite the expectation that, as in HIV, T-cell immunity would be deficient in such patients, cerebrospinal fluid (CSF) immunophenotyping, T-cell activation studies, soluble cytokine mapping and tissue cellular phenotyping demonstrated that patients with s-CNS disease had effective microbiological control, but displayed strong intrathecal expansion and activation of cells of both the innate and adaptive immunity including HLA-DR+ CD4+ and CD8+ cells and NK cells. These expanded CSF T cells were enriched for cryptococcal-antigen specific CD4+ cells and expressed high levels of IFN-γ as well as a lack of elevated CSF levels of typical T-cell specific Th2 cytokines -- IL-4 and IL-13. This inflammatory response was accompanied by elevated levels of CSF NFL, a marker of axonal damage, consistent with ongoing neurological damage. However, while tissue macrophage recruitment to the site of infection was intact, polarization studies of brain biopsy and autopsy specimens demonstrated an M2 macrophage polarization and poor phagocytosis of fungal cells. These studies thus expand the paradigm for cryptococcal disease susceptibility to include a prominent role for macrophage activation defects and suggest a spectrum of disease whereby severe neurological disease is characterized by immune-mediated host cell damage.
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spelling doaj-art-6500928e698640418cf123c97e812eac2025-08-20T02:22:38ZengPublic Library of Science (PLoS)PLoS Pathogens1553-73661553-73742015-05-01115e100488410.1371/journal.ppat.1004884Paradoxical Immune Responses in Non-HIV Cryptococcal Meningitis.Anil A PanackalSimone C WuestYen-Chih LinTianxia WuNannan ZhangPeter KosaMika KomoriAndrew BlakeSarah K BrowneLindsey B RosenFerry HagenJacques MeisStuart M LevitzMartha QuezadoDima HammoudJohn E BennettBibi BielekovaPeter R WilliamsonThe fungus Cryptococcus is a major cause of meningoencephalitis in HIV-infected as well as HIV-uninfected individuals with mortalities in developed countries of 20% and 30%, respectively. In HIV-related disease, defects in T-cell immunity are paramount, whereas there is little understanding of mechanisms of susceptibility in non-HIV related disease, especially that occurring in previously healthy adults. The present description is the first detailed immunological study of non-HIV-infected patients including those with severe central nervous system (s-CNS) disease to 1) identify mechanisms of susceptibility as well as 2) understand mechanisms underlying severe disease. Despite the expectation that, as in HIV, T-cell immunity would be deficient in such patients, cerebrospinal fluid (CSF) immunophenotyping, T-cell activation studies, soluble cytokine mapping and tissue cellular phenotyping demonstrated that patients with s-CNS disease had effective microbiological control, but displayed strong intrathecal expansion and activation of cells of both the innate and adaptive immunity including HLA-DR+ CD4+ and CD8+ cells and NK cells. These expanded CSF T cells were enriched for cryptococcal-antigen specific CD4+ cells and expressed high levels of IFN-γ as well as a lack of elevated CSF levels of typical T-cell specific Th2 cytokines -- IL-4 and IL-13. This inflammatory response was accompanied by elevated levels of CSF NFL, a marker of axonal damage, consistent with ongoing neurological damage. However, while tissue macrophage recruitment to the site of infection was intact, polarization studies of brain biopsy and autopsy specimens demonstrated an M2 macrophage polarization and poor phagocytosis of fungal cells. These studies thus expand the paradigm for cryptococcal disease susceptibility to include a prominent role for macrophage activation defects and suggest a spectrum of disease whereby severe neurological disease is characterized by immune-mediated host cell damage.https://doi.org/10.1371/journal.ppat.1004884
spellingShingle Anil A Panackal
Simone C Wuest
Yen-Chih Lin
Tianxia Wu
Nannan Zhang
Peter Kosa
Mika Komori
Andrew Blake
Sarah K Browne
Lindsey B Rosen
Ferry Hagen
Jacques Meis
Stuart M Levitz
Martha Quezado
Dima Hammoud
John E Bennett
Bibi Bielekova
Peter R Williamson
Paradoxical Immune Responses in Non-HIV Cryptococcal Meningitis.
PLoS Pathogens
title Paradoxical Immune Responses in Non-HIV Cryptococcal Meningitis.
title_full Paradoxical Immune Responses in Non-HIV Cryptococcal Meningitis.
title_fullStr Paradoxical Immune Responses in Non-HIV Cryptococcal Meningitis.
title_full_unstemmed Paradoxical Immune Responses in Non-HIV Cryptococcal Meningitis.
title_short Paradoxical Immune Responses in Non-HIV Cryptococcal Meningitis.
title_sort paradoxical immune responses in non hiv cryptococcal meningitis
url https://doi.org/10.1371/journal.ppat.1004884
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