Treatment of IgG4-related pachymeningitis in a patient with steroid intolerance: The role of early use of rituximab

• Steroids are the treatment of choice for patients with IgG4-related diseases.
• In patients with IgG4-related pachymeningitis, response to MTX can be suboptimal.
• Rituximab has been shown to be effective in patients with IgG4-related pachymeningitis.
• Early RTX treatment should be considered in patients with IgG4-related pachymeningitis.

IgG4-related pachymeningitis is a serious inflammatory condition that can present with symptoms of mass effect and focal deficits. The first-line therapy is steroids and second-line is chemotherapy (methotrexate, azathioprine, etc.). We describe 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.

Figure optionsDownload high-quality image (345 K)Download as PowerPoint slide