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Autoimmune glial fibrillar acid protein astrocytopathy (GFAP) is a new autoimmune meningoencephalomyelitis, affecting meninges, brain, spinal cord and optic nerve, with production of autoantibodies (Anti-GFAP), which can be used as specific biomarkers. Objectives: The work aims to strengthen the relationship between health professionals and this emerging disease that is still underreported in Brazil, describing the clinical and laboratory analyzes, therapeutics and the severity of this pathology and its potentially associated comorbidities. Methodology: This is a narrative review. The keywords “neuroimmunomodulation”, “astrocytes” and “acid fibrillary glial protein”, present in the Descritores em Ciências da Saúde (DeCS), were selected as search strategies in the US National Library of Medicine (PubMed NCBI), in addition to the name of the disease in English (“Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy”). Results: 66 articles were found, 23 of which were selected that included the scope of the study. GFAP astrocytopathy is a rare but underdiagnosed condition. The main findings are subacute headache, myelopathic symptoms, optic disc edema, tremor, ataxia, neck stiffness, autonomic instability, confusion and progressive cognitive impairment. The diagnosis is confirmed by the detection of anti-GFAP antibody (IgG) in cerebrospinal fluid (CSF), this antibody being directly related to astrocytopathy and astrocytic damage. Treatment in the acute stage includes high-dose corticosteroids, intravenous immunoglobulin (IVIG) and plasmapheresis. At a long term, oral corticosteroids and immunosuppressants are used. Conclusion: Although the pathogenesis of the disease is not yet completely known, we observed an affection mainly in meninges, brain, spinal cord and optic nerve, and autoantibodies (AntiGFAP) are directly related to astrocyte damage and can be used as the main biomarker of disease, but it remains underdiagnosed most of the time.

brazilian journal of health review

Investigação clínica-epidemiológica da astrocitopatia por proteína glial fibrilar ácida autoimune: uma revisão narrativa / Clinical-epidemiological investigation of autoimmune glial fibrillar acid protein astrocytopathy: a narrative review