Case Report

A Case of Granulomatosis with Polyangiitis Complicated by Ganciclovir-Resistant Cytomegalovirus Central Nervous System Infection

Abstract

This case report describes a 72-year-old female with a confirmed diagnosis of Granulomatosis with Polyangiitis (GPA), who developed a complex and ultimately fatal clinical course complicated by refractory cytomegalovirus (CMV) meningoencephalitis. Despite initial immunosuppressive treatment for GPA and subsequent antimicrobial therapies for suspected bacterial and fungal infections, the patient presented with recurrent fevers, delirium, and cerebrospinal fluid (CSF) pleocytosis. Repeated CSF analyses and imaging revealed a perplexing picture, with a final diagnosis of CMV infection of the central nervous system (CNS) confirmed by PCR. The infection proved resistant to first-line antiviral therapy with ganciclovir, necessitating the addition of
foscarnet. The patient’s condition deteriorated, culminating in aspiration pneumonia, hemodynamic instability, and death. This case highlights the diagnostic challenges of CNS infections in immunosuppressed patients and the potential for severe, treatment-resistant viral opportunistic infections in the context of GPA and its treatment.

[1] Robson JC, Grayson PC, Ponte C, Suppiah R, Craven A, Judge A, et al. 2022 American College of heumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):315-20. https://doi.org/10.1136/annrheumdis-2021-221795
[2] Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221-32. https://doi.org/10.1056/NEJMoa0909905
[3] Seror R, Mahr A, Ramanoelina J, Pagnoux C, Cohen P, Guillevin L. Central nervous system involvement in Wegener
granulomatosis. Medicine (Baltimore). 2006;85(1):53-65. https://doi.org/10.1097/01.md.0000200166.90373.41
[4] Lurain NS, Chou S. Antiviral drug resistance of human cytomegalovirus. Clin Microbiol Rev. 2010;23(4):689-712.
https://doi.org/10.1128/CMR.00009-10
[5] Roberts DM, Jones RB, Smith RM, Alberici F, Kumaratne DS, Burns S, et al. Rituximab-associated hypogammaglobulinemia: incidence, predictors and outcomes in patients with multisystem autoimmune disease. J Autoimmun. 2015;57:60-5. https://doi.org/10.1016/j.jaut.2014.11.009
[6] Wilson MR, Sample HA, Zorn KC, Arevalo S, Yu G, Neuhaus J, et al. Clinical metagenomic sequencing for diagnosis of meningitis and encephalitis. N Engl J Med. 2019;380(24):2327-40. https:// doi.org/10.1056/NEJMoa1803396
Files
IssueVol 10 No 3 (2025): May-June QRcode
SectionCase Report(s)
DOI https://doi.org/10.18502/crcp.v10i3.20315
Keywords
Granulomatosis with Polyangiitis Cytomegalovirus Meningoencephalitis Ganciclovir Foscarnet Immunosuppression

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Sadidi M, Iranmanesh M, Heydari Havadaragh S. A Case of Granulomatosis with Polyangiitis Complicated by Ganciclovir-Resistant Cytomegalovirus Central Nervous System Infection. CRCP. 2025;10(3):133-137.