====== 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
((Seegobin K, Moustafa MA, Gannon N, Keller K, Hastings J, Gupta V, Tun HW, Jiang L. Successful treatment of IgG4-related hypertrophic pachymeningitis with induction rituximab and dexamethasone followed by maintenance rituximab. Clin Case Rep. 2021 Jan 27;9(3):1610-1614. doi: 10.1002/ccr3.3855. PMID: 33768900; PMCID: PMC7981770.))
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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
((Gospodarev V, Câmara J, Chakravarthy V, Perry A, Wood M, Dietz R, Wang J, De Los Reyes K, Raghavan R. Treatment of IgG4-related pachymeningitis in a patient with steroid intolerance: The role of early use of rituximab. J Neuroimmunol. 2016 Oct 15;299:62-65. doi: 10.1016/j.jneuroim.2016.08.009. Epub 2016 Aug 16. PMID: 27725123.))

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A patient was treated with [[glucocorticoid]], and both the inflammation and patient symptoms were improved
((Hayashi K, Somagawa C, Hayashi Y, Iwanaga M. [A Case of IgG4-related
Hypertrophic Pachymeningitis]. No Shinkei Geka. 2018 Dec;46(12):1103-1109. doi:
10.11477/mf.1436203876. Japanese. PubMed PMID: 30572308.
)).
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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
((Popkirov S, Kowalski T, Schlegel U, Skodda S. Immunoglobulin-G4-related
hypertrophic pachymeningitis with antineutrophil cytoplasmatic antibodies
effectively treated with [[Rituximab]]. J Clin Neurosci. 2015 Jun;22(6):1038-40. doi:
10.1016/j.jocn.2014.12.020. PubMed PMID: 25861887.
)).
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Liao et al.reported a refractory IgG4-related intracranial hypertrophic pachymeningitis that responded to rituximab
((Liao B, Kamiya-Matsuoka C, Fang X, Smith RG. Refractory IgG4-related intracranial hypertrophic pachymeningitis responded to rituximab. Neurol Neuroimmunol Neuroinflamm. 2014 Oct 29;1(4):e41. doi: 10.1212/NXI.0000000000000041. PMID: 25364775; PMCID: PMC4215390.)).