Rituximab for IgG4-related hypertrophic pachymeningitis treatment
A 56-year-old male who had an excellent response to rituximab and dexamethasone after going undiagnosed for 5 years. After 3 years of rituximab maintenance, he has no evidence of disease on brain MRI 1)
Gospodarev et al. described a patient with IgG4-related pachymeningitis in whom steroid use was contraindicated and methotrexate was ineffective. During the course of treatment, the patient presented to the emergency department with receptive and expressive aphasia, slurred speech, right-sided neglect, and loss of sensation. After a single infusion of rituximab and anticonvulsants, her symptoms resolved. Our unique case suggests that patients with IgG4-related pachymeningitis might benefit from early initiation of rituximab 2)
A patient was treated with glucocorticoid, and both the inflammation and patient symptoms were improved 3).
Treatment with Rituximab was initiated which led to disappearance of clinical symptoms and decrease of dural thickening within weeks. This patient presented a possible disease overlap of IgG4-related and ANCA-associated HP and illustrates the effectiveness of Rituximab in refractory IgG4-related HP 4).
Liao et al.reported a refractory IgG4-related intracranial hypertrophic pachymeningitis that responded to rituximab 5).