4.6 Article

Central Nervous System Histoplasma-Associated Post-infectious Inflammatory Response Syndrome (Histo-PIIRS)

Journal

JOURNAL OF CLINICAL IMMUNOLOGY
Volume 41, Issue 3, Pages 545-551

Publisher

SPRINGER/PLENUM PUBLISHERS
DOI: 10.1007/s10875-020-00954-9

Keywords

Fungal meningitis; histoplasma; inflammation; central nervous system

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Funding

  1. NIH Medical Research Scholars Program
  2. NIH
  3. NIH Intramural Research Program

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This case involves a previously healthy adult with hepatitis C presenting with neurological symptoms refractory to antifungal therapy, who showed rapid improvement after initiation of corticosteroid treatment. Elevated CSF cytokines and regional enhancement on brain MRI resolved with corticosteroid therapy, indicating an inflammatory rather than infectious etiology. This is the first documented case of Histoplasma-associated post-infectious inflammatory response syndrome (Histo-PIIRS) showing CSF cytokine reduction in response to corticosteroid therapy.
We present a case of central nervous system (CNS) histoplasmosis in a previously healthy adult with hepatitis C (HCV) presenting with neurological symptoms refractory to antifungal therapy and ventriculoperitoneal (VP) shunting 4 months after initial diagnosis. Persistent symptoms were thought to be inflammatory rather than infectious given negative cerebrospinal fluid (CSF) and serum fungal antigens. The patient promptly improved after initiation of corticosteroid therapy. Elevated CSF cytokines and regional enhancement on brain MRI resolved with corticosteroid treatment. This is the first case of Histoplasma-associated post-infectious inflammatory response syndrome (Histo-PIIRS) documented by CSF cytokine reduction in response to corticosteroid therapy.

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