Abstract Details

Title Treatment of Hypertrophic Pachymeningitis: A Systematic Review

Topic Autoimmune Neurology

Presentation(s) P7 - Poster Session 7 (11:45 AM-12:45 PM)

Poster/Presentation
Number 14-020

Objective To analyze the experiences and perspectives of treatment of Hypertrophic Pachymeningitis (HP).

Background The etiology of HP is variable and is usually largely related to infectious or rheumatological diseases. It is strictly essential to quickly define the etiology to establish adequate treatment.

Design/Methods Were conducted a literature search using the PubMed database, employing the search terms 'hypertrophic pachymeningitis' and 'treatment.' Our selection criteria encompassed Case Reports, Clinical Studies, Clinical Trials, Comparative Studies, Controlled Clinical Trials, Multicenter Studies, Observational Studies, and Randomized Controlled Trials published from the year 2000 to 2023. This rigorous search strategy yielded a total of 117 results. Subsequently, a meticulous screening process based on predefined exclusion criteria was applied, leading to the inclusion of 10 pertinent articles in our study.

Results It is clear that adult treatment, carried out with Rituximab and Dexamethasone, tends to have a good response, with significant improvement leading to the absence of evidence of disease on brain imaging and clinical evolution. Furthermore, it is necessary to identify the etiology involved in the pathophysiological process behind each case, with IgG4 being one of the most cited in the literature. High doses of steroids may be sufficient in earlier situations, and the use of immunosuppressants is widely used. Data indicate a significant reduction in recurrence when comparing steroids with immunosuppressants (42.1% to 40%). For refractory cases, there is a positive description in the literature of Cyclophosphamide, Methotrexate or Mycophenolate.

Conclusions The diagnosis of HP still presents a major challenge, as does the etiological investigation, which defines the treatment. It is essential that experiences between services around the world are exchanged, with the aim of improving the study of basic conditions, in addition to the results obtained with the treatment proposed in each clinical situation.

Authors/Disclosures
BIANCA OLIVEIRA, MD (Horizon Therapeutics) PRESENTER	Dr. OLIVEIRA has nothing to disclose.
Artêmio José Araruna Dias, Sr. (Unifacisa)	Mr. Araruna Dias has nothing to disclose.
Fernando Melo Neto	No disclosure on file
Marinna Karla Viana	No disclosure on file
Yasmim Lisboa	No disclosure on file
Ana Clara L. Rodrigues, MBBS	No disclosure on file
Daniel V. De Siqueira Lima, Jr., MD	Dr. De Siqueira Lima has nothing to disclose.
Davi Guerra	No disclosure on file

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