Background: Ofatumumab, a fully humanized anti-CD20 monoclonal antibody, has shown promising efficacy in limited cases of neuromyelitis optica spectrum disorder, but there is a lack of studies on its use in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. We present a case of refractory GFAP astrocytopathy with poor response to conventional immunosuppressants and rituximab who responded well to subcutaneous ofatumumab.
Case Report: The patient is a 36-year-old woman with a diagnosis of GFAP astrocytopathy and high disease activity. She experienced five relapses over three years despite immunosuppressive treatment with oral prednisone, azathioprine, mycophenolate mofetil, and intravenous rituximab. Additionally, her circulating B cells were not completely depleted during the second administration of rituximab and an allergic reaction occurred. Based on insufficient B cell depletion and allergic reaction to rituximab, subcutaneous ofatumumab was introduced. After twelve injections of ofatumumab without injection-related reactions, she had no further relapses and was sufficiently depleted of the circulating B cells.
Discussion: This case illustrates the effective use and good tolerance of ofatumumab in GFAP astrocytopathy. Further studies are needed to investigate the efficacy and safety of ofatumumab in refractory GFAP astrocytopathy or those intolerant to rituximab.
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http://dx.doi.org/10.3389/fimmu.2023.1164181 | DOI Listing |
J Neurol Sci
December 2024
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Mol Neurobiol
December 2024
Department of Neurology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
Virus encephalitis (VE), recognized as one of the common kinds of central nervous system (CNS) diseases after virus infection, has a surprising correlation with autoimmune encephalitis (AE) when autoimmune antibodies emerge in cerebrospinal fluid (CSF) or serum. Herpes simplex virus and Epstein-Barr virus are the most critical agents worldwide. By molecular mimicry, herpes viruses can invade the brain directly or indirectly.
View Article and Find Full Text PDFFront Immunol
December 2024
Department of Radiology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China.
Objective: Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a novel steroid sensitive autoimmune disease, without a diagnostic consensus. The purpose of this study was to improve early GFAP-A diagnosis by increasing awareness of key clinical characteristics and imaging manifestations.
Methods: Medical records of 13 patients with anti-GFAP antibodies in serum or cerebrospinal fluid (CSF) were reviewed for cross-sectional and longitudinal analysis of clinical and magnetic resonance imaging (MRI) findings.
Exp Cell Res
December 2024
Biophysics and Structural Genomics Division, Saha Institute of Nuclear Physics, A CI of Homi Bhabha National Institute, Kolkata, 700 064, West Bengal, India. Electronic address:
The signaling pathways behind severe astrocytic lysis with Aquaporin4 auto-antibody (AQP4-IgG) seropositivity, and reactive astrocytosis with myelin oligodendrocyte glycoprotein auto-antibody (MOG-IgG) seropositivity, remain largely unexplored in Neuromyelitis optica spectrum disorder (NMOSD), while almost no molecular details being known about double-seronegative (DN) patients. Recent discovery of glial fibrillary acidic protein (GFAP) in DN NMOSD patients' cerebrospinal fluid, akin to AQP4-IgG + ve cases, suggests astrocytopathy. Here, we aim to study small non coding RNA (sncRNA) signature alterations in astrocytes exposed to AQP4-IgG + ve and MOG-IgG + ve patient sera, and their potential resemblance with DN-NMOSD.
View Article and Find Full Text PDFNeurol Neuroimmunol Neuroinflamm
January 2025
From the Departments of Pediatric Neurology (S.S., A.B., K.R.), and Pediatric Radiology (A.P., R.C.), Children's Hospital Datteln, Witten/Herdecke University, Datteln, Germany; Consultant Child Neurologist and Epileptologist at Neoclinic Children's Hospital (V.J.), Jaipur, India; Department of Pediatric Neurology (T.K.), Children's Hospital Datteln, University Witten/Herdecke; Faculty of Health (T.K.), Department of Psychology and Psychotherapy, Chair of Personality Psychology and Diagnosis, Witten/Herdecke University; Center for Paediatric and Adolescent Medicine (U.D.), University Medical Clinic, Mainz; University Children's Hospital Regensburg (KUNO) (T.G.), Hospital St. Hedwig of the Order of St. John, University of Regensburg; Department of Pediatric Neurology (A.N.), VAMED Klinik Geesthacht; Department of Pediatrics (A.N.), University Medical Center Hamburg-Eppendorf; Department of Pediatric Neurology (C.L.-N.), Mutterhaus der Borromäerinnen, Trier; Department of Pediatric Intensive Care (R.A.-H.), University Children's Hal Marburg; Department of Pediatric Neurology (M.F.-B.), Saarland University Medical Center, Homburg/Saar, Germany; Assistance Publique-Hôpitaux de Paris (K.D.), Paris-Saclay University Hospitals, Bicêtre Hospital, Pediatric Neurology Department, National Referral Center for Rare Inflammatory and Auto-immune Brain and Spinal Diseases, Paris Saclay University, France; Neuroimmunology Unit (T.A.), in Sant Joan de Déu Children's Hospital, Esplugues de Llobregat, Barcelona; Neuroimmunology Program (T.A., G.O.-C.), Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic, University of Barcelona; Neurology Unit (G.O.-C.), Hospital Parc Taulí de Sabadell, Sabadell, Barcelona, Spain; Neuroimmunology Laboratory (S.K.), Amrita Institute of Medical Sciences, School of Medicine, Amrita University, Kochi, India; Department of Pediatrics (A.K.); Center for Rare Diseases (A.K.), Faculty of Medicine and University Hospital Cologne, University of Cologne, Germany; Department of Pediatric Neurology (H.M.); Pediatric Neurology Institute (A.F.-V.), Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center; Sackler Faculty of Medicine, Tel Aviv University; Institute of Pediatric Neurology (E.G.-C.), Schneider Children's Medical Center of Israel, Petah Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Israel; University Children's Hospital Oldenburg (H.L.), Department of Neuropediatrics, Oldenburg; Neuropediatric Unit (A.H., R.W.), Karolinska University Hospital and Karolinska Institute Stockholm, Sweden; and Institute of Clinical Chemistry (J.D., F.L.), Neuroimmunology Unit and Department of Neurology, University Medical Center Schleswig-Holstein Campus, Kiel, Germany.
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