Hypertrophic pachymeningitis: a rare manifestation of IgG4 related disease

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dc.contributor.authorSandeepan, K.en_US
dc.contributor.authorJha, M.en_US
dc.contributor.authorPurari, S.en_US
dc.contributor.authorSharma, P. K.en_US
dc.contributor.authorShreyashen_US
dc.contributor.authorKumar, C.en_US
dc.date.accessioned2024-09-24T10:51:34Z
dc.date.available2024-09-24T10:51:34Z
dc.date.issued2023-07
dc.description.abstractHypertrophic pachymeningitis (HP) is a rare form of diffuse inflammatory disease that causes thickening of the dura mater. It can involve the cranial or the spinal dura or both. An increasingly well-known symptom of IgG4-related illness, a fibroinflammatory syndrome that may affect almost any organ, is IgG4-related hypertrophic pachymeningitis (IgG4-RHP). It is estimated that IgG4-RHP may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. Contrast magnetic resonance imaging (MRI) shows pachymeningeal enhancement. Serum IgG4 levels may be elevated but are normal in most patients. However, most patients have elevated cerebrospinal fluid (CSF) IgG4 index. Hence, CSF IgG4 index could serve as a less invasive diagnostic marker of IgG4-RHP. Confirmation of diagnosis is by meningeal biopsy that shows swirling “storiform” fibrosis with lymphocytic infiltrates, obliterate phlebitis and IgG4 positive plasma cells. This case highlights the diagnostic dilemma of IgG4-RHP as gold standard of diagnosis is meningeal biopsy which has many of its own limitations. CSF IgG4 index could be an alternate option for meningeal biopsy when the procedure is contraindicated or uninformative.en_US
dc.identifier.affiliationsDepartment of Medicine, Patna Medical College and Hospital, Patna, Bihar, Indiaen_US
dc.identifier.affiliationsSadar Hospital, Madhepura, Bihar, Indiaen_US
dc.identifier.affiliationsDepartment of Medicine, Patna Medical College and Hospital, Patna, Bihar, Indiaen_US
dc.identifier.affiliationsDepartment of Medicine, Patna Medical College and Hospital, Patna, Bihar, Indiaen_US
dc.identifier.affiliationsDepartment of Medicine, Patna Medical College and Hospital, Patna, Bihar, Indiaen_US
dc.identifier.affiliationsDepartment of Medicine, Patna Medical College and Hospital, Patna, Bihar, Indiaen_US
dc.identifier.citationSandeepan K., Jha M., Purari S., Sharma P. K., Shreyash, Kumar C.. Hypertrophic pachymeningitis: a rare manifestation of IgG4 related disease. International Journal of Research in Medical Sciences. 2023 Jul; 11(7): 2732-2735en_US
dc.identifier.issn2320-6071
dc.identifier.issn2320-6012
dc.identifier.placeIndiaen_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/234517
dc.languageenen_US
dc.publisherMedip Academyen_US
dc.relation.issuenumber7en_US
dc.relation.volume11en_US
dc.source.urihttps://doi.org/10.18203/2320-6012.ijrms20232134en_US
dc.subjectHypertrophic pachymeningitisen_US
dc.subjectIgG4 related diseaseen_US
dc.subjectCSF IgG4 indexen_US
dc.subjectStoriform fibrosisen_US
dc.titleHypertrophic pachymeningitis: a rare manifestation of IgG4 related diseaseen_US
dc.typeJournal Articleen_US
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