IgG4-Related Hypertrophic Pachymeningitis: A Great Clinical Imitator

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Manuel Alejandro Vega Villegas, M.D.
Daniel Vidrio Carrizales, M.D
Siomara Estefanía Luquin Ruiz, M.D.
Mónica Susana García Bravo, M.D.

Abstract

We present a diagnostically challenging case of a 45-year-old woman with a long-standing clinical course characterized by episodic dysphonia, epistaxis, seizures, and cranial neuropathies. Extensive evaluations initially led to a suspected diagnosis of non-Hodgkin lymphoma. However, elevated serum IgG4 levels and histopathological reevaluation confirmed IgG4-related hypertrophic pachymeningitis. The patient responded well to methotrexate and prednisone. This case underscores the importance of considering IgG4-RD in unexplained meningeal syndromes and highlights the need for timely recognition to avoid invasive interventions.

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How to Cite
Vega Villegas, M.D., Manuel Alejandro, Daniel Vidrio Carrizales, Siomara Estefanía Luquin Ruiz, and Mónica Susana García Bravo. 2025. “IgG4-Related Hypertrophic Pachymeningitis: A Great Clinical Imitator”. International Journal of Medical Science and Clinical Research Studies 5 (6):941-42. https://doi.org/10.47191/ijmscrs/v5-i06-21.
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References

I. Wallace ZS, Naden RP, Chari S, et al. The 2019 ACR/EULAR Classification Criteria for IgG4-Related Disease. Arthritis Rheumatol. 2020;72(1):7-19. DOI: 10.1002/art.41120

II. Lu LX, Della-Torre E, Stone JH, Clark SW. IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 2014;71(6):785-793.

DOI: 10.1001/jamaneurol.2014.243

III. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539-551. DOI: 10.1056/NEJMra1104650

IV. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on pathology of IgG4-RD. Mod Pathol. 2012;25(9):1181–1192. DOI:10.1038/modpathol.2012.72

V. Carruthers MN, Khosroshahi A, Augustin T, et al. The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis. 2015;74(1):14–18.

DOI:10.1136/annrheumdis-2013-203929

VI. Ryu JA, Moon J, Kim SH, et al. Atypical presentation of IgG4-related hypertrophic pachymeningitis mimicking infectious meningoencephalitis. BMC Neurol. 2020;20(1):238. DOI:10.1186/s12883-020-01843-2

VII. Khosroshahi A, Carruthers MN, Deshpande V, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore). 2012;91(1):57–66. DOI:10.1097/MD.0b013e3182431ef6

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