Glial fibrillary acidic protein immunoglobulin <scp>G</scp> as biomarker of autoimmune astrocytopathy: Analysis of 102 patients

Flanagan, Eoin P.; Hinson, Shannon R.; Lennon, Vanda A.; Fang, Boyan; Aksamit, Allen J.; Morris, Pearse; Basal, Eati; Honorat, Josephe A.; Alfugham, Nora; Linnoila, Jenny; Weinshenker, Brian G.; Pittock, Sean J.; McKeon, Andrew

doi:10.1002/ana.24881

Cited by 438 publications

(737 citation statements)

References 34 publications

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“…Necrotizing meningoencephalitis and granulomatous meningoencephalitis are idiopathic inflammatory diseases of the central nervous system associated with GFAP antibodies in dogs, demonstrating inflammatory cell infiltration of the perivascular area and brain parenchyma by mixed populations of CD3 + T cells, B lymphocytes, plasma cells, and macrophages in almost equal ratios [7, 8]. Our current results support the hypothesis that T lymphocytes interact with B cells and other inflammatory components of the immune system (microglia, macrophages, and antibody secretion by plasma cells), contributing to the pathogenesis of GFAP neurological autoimmunity [2]. …”

Section: Discussionsupporting

confidence: 77%

Brain Immunohistopathology in a Patient with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy

Shu

Long

Chang

et al. 2018

Neuroimmunomodulation

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Background: Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel meningoencephalomyelitis. However, the pathogenesis of this disease is unclear. We therefore examined a brain biopsy from a patient with autoimmune GFAP astrocytopathy by immunohistopathology. Methods: We examined brain biopsy sections from a patient with autoimmune GFAP astrocytopathy using hematoxylin and eosin (HE) and Luxol fast blue (LFB) staining, and immunostaining with antibodies for CD4, CD8, CD3, CD20, CD68, CD138, Neu-N, GFAP, myelin oligodendrocyte glycoprotein (MOG), and aquaporin-4 (AQP4). Results: HE staining revealed extensive inflammatory cells (marked lymphocytes) around brain vessels, and LFB showed no signs of demyelination or axon loss. Immunohistochemical analysis showed CD3⁺ and CD4⁺ T cells cuffing around brain vessels, accompanied by CD8⁺ T cells, CD20⁺ B cells, and CD138⁺ plasma cells, while some macrophages (CD68⁺) were scattered throughout the brain parenchyma. There was no loss of AQP4 or MOG expression in this patient, while GFAP was abundantly expressed. Conclusions: These findings suggest that inflammatory cells, including T cells, B cells, plasma cells, and macrophages, are involved in autoimmune GFAP astrocytopathy. Demyelination and astrocyte loss may not necessarily occur in this disease.

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Section: Discussionsupporting

confidence: 77%

Brain Immunohistopathology in a Patient with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy

Shu

Long

Chang

et al. 2018

Neuroimmunomodulation

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“…In the present study, we described 7 patients with GFAP antibodies in their CSF, which has been defined as GFAP astrocytopathy in recent reports [1,2] . Although this study had a small sample number, we are the first to describe the long-term treatment and evolution in Chinese patients.…”

Section: Discussionmentioning

confidence: 75%

“…The Mayo clinic study reported that patients with GFAP astrocytopathy responded well to steroids or aggressive immunosuppression [1,2] . However, evidencebased therapy of GFAP astrocytopathy is absent.…”

Section: Discussionmentioning

confidence: 99%

“…However, evidencebased therapy of GFAP astrocytopathy is absent. In the Mayo study [2] , long-term treatment details were only available for 9 patients. Three of these had coexisting anti-NMDAR encephalitis, treated with oral prednisone, MMF, or azathioprine.…”

Section: Discussionmentioning

confidence: 99%

“…in animals or humans with this disease [1][2][3][4] . A human study at the Mayo Clinic showed that 13 patients responded well to initial intravenous high-dose steroid treatment, but 7 patients experienced relapse during dose tapering.…”

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confidence: 99%

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Treatment of Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: Follow-Up in 7 Cases

Yang¹,

Liang²,

Huang³

et al. 2017

Neuroimmunomodulation

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Objective: The aim of this work was to report an autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy with long-term follow-up in 7 patients. Methods: Antibodies were detected by indirect immunofluorescence assay and patient data were analyzed retrospectively. Results: Seven patients (4 female, 3 male) with ≥1 year follow-up were included. All patients had positive GFAP antibodies in their cerebral spinal fluid (CSF). Their median age at disease onset was 56 years (range 27-69) and the median disease duration was 1 year (range 1-4). In the initial attack, all patients received intravenous methylprednisolone (IVMP) followed by oral steroids, which were tapered. Six patients received intravenous immunoglobulin (IVIg). One patient had no response to IVIg and IVMP. Four patients received immunosuppressive agents. Five patients underwent the second lumbar puncture after treatment. CSF white blood cell counts, protein levels, and antibody titers were significantly decreased. CSF protein levels correlated positively with the Expanded Disability Status Scale score, which was elevated at each lumbar puncture. Four patients experienced relapse. To date, 6 patients had a bad prognosis, of which 2 died. Conclusions: Some patients with GFAP astrocytopathy experienced a poor response to treatment although they received steroids and immunosuppressive agents.

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Novel Glial Targets and Recurrent Longitudinally Extensive Transverse Myelitis

et al. 2018

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Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients

Abstract: GFAPα-IgG, when detected in CSF, is highly specific for an immunotherapy-responsive autoimmune CNS disorder, sometimes with paraneoplastic cause. Ann Neurol 2017;81:298-309.

Cited by 438 publications

References 34 publications

Brain Immunohistopathology in a Patient with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy

Brain Immunohistopathology in a Patient with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy

Treatment of Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy: Follow-Up in 7 Cases

Novel Glial Targets and Recurrent Longitudinally Extensive Transverse Myelitis

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