A clinical approach to hypertrophic pachymeningitis

ABSTRACT

Importance: Hypertrophic pachymeningitis (HP) is a non-usual manifestation of rheumatologic, infectious, and neoplastic diseases. Etiological diagnosis is a challenge, but when made promptly it creates a window of opportunity for treatment, with the possibility of a total reversal of symptoms. Observations: HP is an inflammatory process of the dura mater that can occur as a manifestation of sarcoidosis, granulomatosis with polyangiitis, and IgG4-related disease. The HP case evaluation is extensive and includes central nervous system imaging, cerebrospinal fluid analysis, serology, rheumatologic tests, and systemic survey for other manifestations sites. After systemic investigation, meningeal biopsy might be necessary. Etiology guides HP treatment, and autoimmune disorders are treated with corticosteroids alone or associated with an immunosuppressor. Conclusion: HP is a manifestation of several diseases, and a precise etiological diagnosis is crucial because of the difference among treatments. An extensive investigation of patients with HP helps early diagnosis and correct treatment.

RESUMO

Importância: Paquimeningite hipertrófica (PH) é uma manifestação não usual de doenças reumatológicas, infecciosas e neoplásicas. O diagnóstico etiológico por vezes é um desafio, entretanto quando realizado em tempo cria uma janela de tratamento com a possibilidade de reversão total dos sintomas. Observações: A PH é um processo inflamatório da dura-máter que pode ocorrer como manifestação da sarcoidose, granulomatose com poliangeíte e doença relacionada à IgG4. A avaliação dos casos de PH é extensa e inclui imagem do sistema nervoso central, análise de líquor, sorologias, provas reumatológicas e rastreio sistêmico para doença em outros sítios. Por vezes, após toda a investigação sistêmica, a biópsia de meninge é necessária. A etiologia orienta o tratamento da HP, sendo que em doenças autoimunes adota-se o uso de corticosteroides isolados ou associados a um imunossupressor. Conclusão e Relevância: A PH é uma manifestação de várias doenças, e seu diagnóstico etiológico preciso é fundamental, visto a diferença entre os possíveis tratamentos. Uma investigação ampla nos casos de PH ajuda no diagnóstico precoce e tratamento adequado.

Keywords:

Pachymeningitis - Granulomatosis with Polyangiitis - Immunoglobulin G4-Related Disease - Sarcoidosis

Palavras-chave:

Paquimeningite - Granulomatose com Poliangiite - Doença Relacionada a Imunoglobulina G4 - Sarcoidose

Publikationsverlauf

Eingereicht: 05. April 2020

Angenommen: 14. Mai 2020

Artikel online veröffentlicht:
07. Juni 2023

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