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Sarcoidosis with cryptococcal infection apparently engaging only immune-privileged body compartments: a case report
BACKGROUND: Infection with the Cryptococcus neoformans yeast fungus is largely restricted to patients with HIV, sarcoidosis or immunosuppressive therapies. In sarcoidosis, there is intense local immune response in granuloma lesions, coupled with a paradoxical systemic anergy. An analysis of cryptoco...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7310135/ https://www.ncbi.nlm.nih.gov/pubmed/32571242 http://dx.doi.org/10.1186/s12879-020-05174-5 |
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author | van der Stoep, Johannes H. Sigstad, Eva Bredberg, Anders |
author_facet | van der Stoep, Johannes H. Sigstad, Eva Bredberg, Anders |
author_sort | van der Stoep, Johannes H. |
collection | PubMed |
description | BACKGROUND: Infection with the Cryptococcus neoformans yeast fungus is largely restricted to patients with HIV, sarcoidosis or immunosuppressive therapies. In sarcoidosis, there is intense local immune response in granuloma lesions, coupled with a paradoxical systemic anergy. An analysis of cryptococcal infection in sarcoidosis may therefore shed light on whether opportunistic pathogens preferentially engage immune-privileged tissues. CASE PRESENTATION: A 54-year-old man was admitted to our hospital after 2 months with palpitations and activity-related presyncope. A chest X-ray was normal, electrocardiography showed type-II atrioventricular-block, and there was a tentative diagnosis of myocarditis. Computed tomography reported minor hilar lymph glands and multiple nodular lesions in the lungs. Magnetic resonance imaging showed oedema and nodular structures in the heart, and fibrosis and granulomas were found in endomyocardial biopsies. The diagnosis was revised to cardiac sarcoidosis, and prednisone was initiated. In parallel, positron-emission tomography had revealed a marked uptake in the right thyroid lobe, a thyroid lobectomy was then performed, and the pathology showed a BRAF-positive papillary thyroid carcinoma. Four days postoperatively the patient developed symptoms suggestive of meningoencephalitis. Cerebrospinal fluid and blood cultures yielded growth of C. neoformans. Fungal staining of the thyroid specimen revealed cryptococcal elements in the carcinoma and in granulomas close to the tumour. Notably, there was no evidence of cryptococci in the heart sarcoid sections or in the normal thyroid parenchyma. The patient was successfully treated with antifungal agents and at the 2-year follow-up there was no evidence of thyroid cancer relapse. CONCLUSION: This sarcoidosis patient had a remarkable clinic with evidence of cryptococcal infection only in body compartments commonly regarded to be immune-privileged. The findings suggest that an opportunistic and environmentally abundant pathogen, when infecting an immunocompromised host, primarily engages immunodeficient locations such as the brain, a tumour microenvironment and some forms of granuloma. |
format | Online Article Text |
id | pubmed-7310135 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-73101352020-06-23 Sarcoidosis with cryptococcal infection apparently engaging only immune-privileged body compartments: a case report van der Stoep, Johannes H. Sigstad, Eva Bredberg, Anders BMC Infect Dis Case Report BACKGROUND: Infection with the Cryptococcus neoformans yeast fungus is largely restricted to patients with HIV, sarcoidosis or immunosuppressive therapies. In sarcoidosis, there is intense local immune response in granuloma lesions, coupled with a paradoxical systemic anergy. An analysis of cryptococcal infection in sarcoidosis may therefore shed light on whether opportunistic pathogens preferentially engage immune-privileged tissues. CASE PRESENTATION: A 54-year-old man was admitted to our hospital after 2 months with palpitations and activity-related presyncope. A chest X-ray was normal, electrocardiography showed type-II atrioventricular-block, and there was a tentative diagnosis of myocarditis. Computed tomography reported minor hilar lymph glands and multiple nodular lesions in the lungs. Magnetic resonance imaging showed oedema and nodular structures in the heart, and fibrosis and granulomas were found in endomyocardial biopsies. The diagnosis was revised to cardiac sarcoidosis, and prednisone was initiated. In parallel, positron-emission tomography had revealed a marked uptake in the right thyroid lobe, a thyroid lobectomy was then performed, and the pathology showed a BRAF-positive papillary thyroid carcinoma. Four days postoperatively the patient developed symptoms suggestive of meningoencephalitis. Cerebrospinal fluid and blood cultures yielded growth of C. neoformans. Fungal staining of the thyroid specimen revealed cryptococcal elements in the carcinoma and in granulomas close to the tumour. Notably, there was no evidence of cryptococci in the heart sarcoid sections or in the normal thyroid parenchyma. The patient was successfully treated with antifungal agents and at the 2-year follow-up there was no evidence of thyroid cancer relapse. CONCLUSION: This sarcoidosis patient had a remarkable clinic with evidence of cryptococcal infection only in body compartments commonly regarded to be immune-privileged. The findings suggest that an opportunistic and environmentally abundant pathogen, when infecting an immunocompromised host, primarily engages immunodeficient locations such as the brain, a tumour microenvironment and some forms of granuloma. BioMed Central 2020-06-22 /pmc/articles/PMC7310135/ /pubmed/32571242 http://dx.doi.org/10.1186/s12879-020-05174-5 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report van der Stoep, Johannes H. Sigstad, Eva Bredberg, Anders Sarcoidosis with cryptococcal infection apparently engaging only immune-privileged body compartments: a case report |
title | Sarcoidosis with cryptococcal infection apparently engaging only immune-privileged body compartments: a case report |
title_full | Sarcoidosis with cryptococcal infection apparently engaging only immune-privileged body compartments: a case report |
title_fullStr | Sarcoidosis with cryptococcal infection apparently engaging only immune-privileged body compartments: a case report |
title_full_unstemmed | Sarcoidosis with cryptococcal infection apparently engaging only immune-privileged body compartments: a case report |
title_short | Sarcoidosis with cryptococcal infection apparently engaging only immune-privileged body compartments: a case report |
title_sort | sarcoidosis with cryptococcal infection apparently engaging only immune-privileged body compartments: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7310135/ https://www.ncbi.nlm.nih.gov/pubmed/32571242 http://dx.doi.org/10.1186/s12879-020-05174-5 |
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