Intrathecal rituximab in immunoglobulin G4-hypertrophic pachymeningitis

Soares, Carolina; Martins, Diana; Pestana-Silva, Roberto; Honavar, Mrinalini; Faria, Olinda; Abreu, Pedro; Costa, Andreia

doi:10.1016/j.jneuroim.2019.576997

Cited by 3 publications

(3 citation statements)

References 13 publications

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“…Two reports in the literature describe successful treatment of IgG4RD-HP with intrathecal rituximab after failure of intravenous rituximab ( 2 , 3 ). In both cases, the patients had frontal pachymeningitis, a location that affords lower surgical risk, allowing for biopsy confirmation.…”

Section: Discussionmentioning

confidence: 99%

“…Both received intrathecal rituximab < 3 years after diagnosis and demonstrated marked improvement in pachymeningeal enhancement and associated parenchymal T2 hyperintensities. One of the cases also described normalization of CSF:serum albumin ratio (QQ alb ), a measure of CSF flow rate and blood-CSF barrier dysfunction ( 2 , 9 ). Neither measured CSF IgG4 markers or described adverse effects from intrathecal rituximab.…”

Section: Discussionmentioning

confidence: 99%

“…Intravenous rituximab is a second-line therapy but has limited ability to penetrate the blood-brain barrier. Intrathecal rituximab has been reported to lead to clinical improvement in IgG4-RD-HP ( 2 , 3 ). We report a patient with IgG4RD-HP who developed hearing loss, optic neuropathy, and hydrocephalus and experienced clinicoradiographic improvement on intrathecal rituximab after failure of intravenous rituximab.…”

Section: Introductionmentioning

confidence: 99%

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Treatment of IgG4-related disease-associated hypertrophic pachymeningitis with intrathecal rituximab: a case report

et al. 2023

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IgG4-related disease-associated hypertrophic pachymeningitis (IgG4RD-HP) is a fibroinflammatory autoimmune disorder in which diagnosis is difficult without biopsy. Guidance on management of disease refractory to glucocorticoids and intravenous rituximab is limited. We present the case of a 68-year-old woman with IgG4RD-HP who developed sensorineural hearing loss with associated bulky basilar pachymeningeal enhancement. Her cerebrospinal fluid was inflammatory and had an elevated IgG4 concentration, strongly suggestive of IgG4RD-HP. Biopsy of involved meninges was not possible due to surgical risk. Over years she developed bilateral optic neuropathies and hydrocephalus, requiring intravenous rituximab and ventriculoperitoneal shunt. Her disease was refractory to glucocorticoids. Despite maintenance intravenous rituximab, she developed slowly progressive symptoms of intracranial hypertension and hydrocephalus with persistently inflammatory spinal fluid. Switching to intrathecal rituximab therapy led to dramatic improvement in gait and headache and reduced pachymeningeal bulk and metabolic activity. In patients with IgG4RD-HP refractory to glucocorticoids and intravenous rituximab, intrathecal rituximab may be an efficacious therapy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%