IgG4-related hypertrophic pachymeningitis treatment [Neurosurgery Wiki]

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====== IgG4-related hypertrophic pachymeningitis treatment ======


The diagnosis of [[IgG4-related hypertrophic pachymeningitis]] is challenging but is of great relevance as treatment differs significantly from other forms of pachymeningitis and a specific therapeutic approach may avoid long-term neurological complications
((Esmaeilzadeh M, Dadak M, Atallah O, Möhn N, Skripuletz T, Hartmann C, Banan R, Krauss JK. IgG4-related hypertrophic pachymeningitis with tumor-like intracranial and intracerebral lesions. Acta Neurochir (Wien). 2022 Aug 17. doi: 10.1007/s00701-022-05340-5. Epub ahead of print. PMID: 35974231.)).

There is no specific treatment protocol for IgG4 related pachymeningitis
((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.)).

High-dose steroids are the initial treatment of choice; other immunosuppressive agents have shown variable efficacy in reducing meningeal hypertrophy and mostly used in disease relapse setting
((Lu LX, Della-Torre E, Stone JH, Clark SW. IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 2014 Jun;71(6):785-93. doi: 10.1001/jamaneurol.2014.243. PMID: 24733677.))

Second-line is chemotherapy [[(methotrexate]], [[azathioprine]], etc.). 
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Surgery is necessary when lesions progress and patients start to develop cranial nerve function deficit
((Yu Y, Lv L, Yin SL, Chen C, Jiang S, Zhou PZ. Clivus-involved immunoglobulin G4 related hypertrophic pachymeningitis mimicking meningioma: A case report. World J Clin Cases. 2022 Jun 26;10(18):6269-6276. doi: 10.12998/wjcc.v10.i18.6269. PMID: 35949844; PMCID: PMC9254204.)).

===== Rituximab =====

see [[Rituximab for IgG4-related hypertrophic pachymeningitis treatment]].