title: Treatment of IgG4-related pachymeningitis in a patient with steroid intolerance: The role of early use of rituximab.
abstract: 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.
authors.affiliation: Loma Linda University School of Medicine, 11175 Campus St., Loma Linda, 92350, CA, United States.. Electronic address: vgospodarev@llu.edu.
authors.forename: Vadim
authors.initials: V
authors.lastname: Gospodarev
authors.affiliation: Loma Linda University School of Medicine, 11175 Campus St., Loma Linda, 92350, CA, United States.. Electronic address: jcamara@llu.edu.
authors.forename: Justin
authors.initials: J
authors.lastname: Câmara
authors.affiliation: Neurosurgery, Loma Linda University School of Medicine, Loma Linda, CA, United States. Electronic address: vchakravarthy@llu.edu.
authors.forename: Vikram
authors.initials: V
authors.lastname: Chakravarthy
authors.affiliation: Pathology, UCSF, San Francisco, CA, United States. Electronic address: Arie.Perry@ucsf.edu.
authors.forename: Arie
authors.initials: A
authors.lastname: Perry
authors.affiliation: Pathology, UCSF, San Francisco, CA, United States. Electronic address: matthew.wood@ucsf.edu.
authors.forename: Matthew
authors.initials: M
authors.lastname: Wood
authors.affiliation: Pathology, Loma Linda University School of Medicine, Loma Linda, CA, United States. Electronic address: rdietz@llu.edu.
authors.forename: Robin
authors.initials: R
authors.lastname: Dietz
authors.affiliation: Pathology, Loma Linda University School of Medicine, Loma Linda, CA, United States. Electronic address: jwang@llu.edu.
authors.forename: Jun
authors.initials: J
authors.lastname: Wang
authors.affiliation: Neurosurgery, Loma Linda University School of Medicine, Loma Linda, CA, United States. Electronic address: KDelosreyes@llu.edu.
authors.forename: Kenneth
authors.initials: K
authors.lastname: De Los Reyes
authors.affiliation: Pathology, Loma Linda University School of Medicine, Loma Linda, CA, United States. Electronic address: rraghavan@llu.edu.
authors.forename: Ravi
authors.initials: R
authors.lastname: Raghavan
mesh_terms: D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007155:Immunologic Factors / Q000008:administration & dosage; D008581:Meningitis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000276:immunology*; D008875:Middle Aged; D000069283:Rituximab / Q000008:administration & dosage*; D013256:Steroids / Q000009:adverse effects; D016896:Treatment Outcome
chemical_list: D007074:Immunoglobulin G; D007155:Immunologic Factors; D013256:Steroids; D000069283:Rituximab
keywords: IgG4-related diseases; IgG4-related pachymeningitis; methotrexate; rituximab