Artêmio Dias1, Fernando Melo Neto2, Marinna Viana3, Yasmim Barbosa2, Ana Clara Rodrigues4, Daniel De Siqueira Lima5, Davi Guerra4, Bianca Etelvina4
1Centro Universitário UNIFACISA, 2Centro Universitário de João Pessoa (UNIPÊ), 3Universidade Federal de Minas Gerais (UFMG), 4Fundação Centro Integrado de Apoio à Pessoa com Deficiência (FUNAD), 5CREM PB
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.