Abstract
Introduction: The present report presents a case of chronic hypertrophic pachymeningitis secondary to IgG4-related disease diagnosed in southwestern Colombia, confirmed by meningeal biopsy, immunohistochemical markers and elevated serum levels of IgG4. These paraclinical findings had not been previously reported in the local literature, making them relevant to the medical community in our region.
Case presentation: A 56-year-old male patient came to the emergency room for a two-month history of holocranial headache associated with nausea, emesis, progressive hearing loss, weight loss, and unquantified temperature increases. Within the etiological study, brain MRI revealed thickening and diffuse pachymeningeal enhancement, infectious, neoplastic pathologies and systemic autoimmune diseases were ruled out, and in the absence of a diagnosis, a meningeal biopsy was performed, which finally reported histomorphological findings compatible with sclerosing disease related to IgG4. The patient presented progressive clinical improvement with the initiation of systemic steroids.
Discussion: There are different criteria for the diagnosis or classification of ER-IgG4, in which a constant is to demonstrate clinical and/or radiological evidence of involvement of at least one organ added to the histological criteria. The mainstay of treatment is steroids in intermediate or high doses with subsequent progressive tapering, relapses are not uncommon, however, there are more and more immunomodulatory medications that can reduce or replace the use of steroids in case of intolerance or refractoriness.
Conclusions: Intracranial ER-IgG4 involvement is rare, therefore it is important to know its clinical presentation to suspect it and treat it promptly, avoiding fatal consequences for the patient.
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