Invasive Pulmonary Aspergillosis with Marked Eosinophilia as a Unique Presentation of Chronic Granulomatous Disease
  • Julie Van Woensel
    Department of Pulmonology, Zuyderland Medical Center, Heerlen, the Netherlands
  • Remy Mostard
    Department of Clinical Chemistry & Hematology, Zuyderland Medical Center, Heerlen, the Netherlands
  • Mathie Leers
    Department of Clinical Chemistry & Hematology, Zuyderland Medical Center, Heerlen, the Netherlands

Keywords

Pulmonary aspergillosis, chronic granulomatous disease, hypereosinophilia, eosinophilic pneumonia, pulmonary infiltrates with eosinophilia

Abstract

Background: Chronic granulomatous disease (CGD) is a rare immunodeficiency disorder resulting in phagocytic cell dysfunction. It is characterized by deficient cellular immunity against bacteria and fungi, and an excessive inflammatory response resulting in granuloma formation. It manifests, usually in early childhood, with recurrent bacterial and fungal infections or inflammatory complications. The infections, such as invasive pulmonary aspergillosis, can be life-threatening. 

Case description: Our patient was a 40-year-old man with no pulmonary history who presented with bilateral pulmonary nodules and pronounced eosinophilia in peripheral blood and bronchoalveolar lavage fluid, mimicking eosinophilic pneumonia. During treatment with corticosteroids, the patient deteriorated clinically and radiographically. Extensive investigations failed to provide a diagnosis. A lung biopsy demonstrated the presence of granulomas and Aspergillus fumigatus hyphae. Advanced screening to detect underlying immunodeficiency revealed CGD. 

Discussion: This case report describes a unique first presentation of CGD. It reminds physicians of the possibility of CGD as an underlying immune disorder in invasive aspergillosis and highlights the challenges of diagnosing invasive pulmonary aspergillosis. We discuss the diagnostic pitfalls of this case and propose a diagnostic work-up for eosinophilic lung disease. 

VIEW THE ENTIRE ARTICLE

References

  • Seger R. Chronic granulomatous disease 2018: advances in pathophysiology and management. LymphoSign Journal 2019;6:1–16.
  • Knox BP, Huttenlocher A, Keller NP. Real-time visualization of immune cell clearance of Aspergillus fumigatus spores and hyphae. Fungal Genet Biol 2017;105:52–54.
  • Soler-Palacín P, de Gracia J, González-Granado LI, Martín C, Rodríguez-Gallego C, Sánchez-Ramón S, et al. Primary immunodeficiency in lung disease: warning signs, diagnosis and management. Respir Res 2018;19:1–13.
  • Williams D, Kadaria D, Sodhi A, Fox R, Williams G, Threlkeld S. Chronic granulomatous disease presenting as Aspergillus fumigatus pneumonia in a previously healthy young woman. Am J Case Rep 2017;18:351–354.
  • Malacco NLSO, Rachid MA, Gurgel ILS, Moura TR, Sucupira PHF, de Sousa LP, et al. Eosinophil-associated innate IL-17 response promotes Aspergillus fumigatus lung pathology. Front Cell Infect Microbiol 2019;8:1–13.
  • Labrosse R, Abou-Diab J, Blincoe A, Cros G, Luu TM, Deslandres C, et al. Very early-onset inflammatory manifestations of X-linked chronic granulomatous disease. Front Immunol 2017;8:1–7.
  • Meersseman W, Lagrou K, Maertens J, Wilmer A, Hermans G, Vanderschueren S, et al. Galactomannan in bronchoalveolar lavage fluid: a tool for diagnosing aspergillosis in intensive care unit patients. Am J Respir Crit Care Med 2008;177:27–34.
  • Zou M, Tang L, Zhao S. Systematic review and meta-analysis of detecting galactomannan in bronchoalveolar lavage fluid for diagnosing invasive aspergillosis. PLoS One 2012;7(8):e43347. doi: 10.1371/journal.pone.0043347
  • Guo YL, Chen YQ, Wang K, Qin SM, Wu C, Kong JL. Accuracy of BAL galactomannan in diagnosing invasive aspergillosis: a bivariate meta-analysis and systematic review. Chest 2010;138:817–824. doi: 10.1378/chest.10-0488
  • Meersseman W, Vandecasteele SJ, Wilmer A, Verbeken E, Peetermans WE, Van Wijngaerden E. Invasive aspergillosis in critically ill patients without malignancy. Am J Respir Crit Care Med 2004;170:621–625.
  • Cottin V. Eosinophilic lung diseases. Clin Chest Med 2016;37(3):535–556.
  • Views: 482
    HTML downloads: 71
    PDF downloads: 242


    Published: 2022-07-11
    Issue: 2022: Vol 9 No 7 (view)


    How to cite:
    1.
    Van Woensel J, Mostard R, Leers M. Invasive Pulmonary Aspergillosis with Marked Eosinophilia as a Unique Presentation of Chronic Granulomatous Disease. EJCRIM 2022;9 doi:10.12890/2022_003423.

    Most read articles by the same author(s)