Successful treatment of HIV-related progressive multifocal leukoencephalopathy and immunological reconstitution inflammatory syndrome with intravenous human immunoglobulin: a case report.

Abstract

Background: Progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome (PML-IRIS) is a high-mortality disease among patients with AIDS. It is caused by infection with the John Cunningham virus (JCV). Currently, there are no specific antiviral treatments targeting JCV. Thus, immune reconstitution remains the primary therapeutic approach.

Case presentation: A 29-year-old male patient diagnosed with AIDS presented for medical evaluation after two months of antiretroviral therapy (ART), reporting symptoms of dizziness and headache. The detection of JC virus was confirmed in cerebrospinal fluid (CSF) through metagenomic next-generation sequencing (mNGS) analysis. Plain and enhanced cranial MRI scans revealed diffusely distributed nodular and patchy enhancement shadows within the brain parenchyma, consistent with a diagnosis of PML-IRIS. Given that glucocorticoids and PD-1 inhibitors may possess higher toxicity profiles and side effects compared to intravenous immunoglobulin (IVIG), which has been shown to restore immune function while causing fewer adverse reactions rapidly, a five-day regimen of intravenous IVIG infusion was administered in conjunction with continuous ART. Following this intervention, the patient showed significant clinical improvement, including reduced dizziness and headache, and improved neurological function.

Conclusions: The administration of IVIG alone may be considered an effective immunologic reconstitution strategy in treating early stages of PML-IRIS associated with AIDS, despite the complexity of the disease. This approach could be attributed to direct anti-JCV effects, neutralization of toxins, inhibition of inflammatory cytokine release, and its relatively tolerable safety profile. This case report aims to serve as a reference for clinical practitioners regarding the use of standalone IVIG therapy for HIV-related early PML-IRIS management; however, further investigation is warranted to determine its efficacy in cases where PML-IRIS has been detected at later stages.