title: Immunoglobulin-G4-related hypertrophic pachymeningitis with antineutrophil cytoplasmatic antibodies effectively treated with rituximab.
abstract: We describe a 52-year-old man with hypertrophic pachymeningitis (HP) who was both seropositive for antineutrophil cytoplasmatic antibodies (ANCA) against myeloperoxidase, and had an immunoglobulin G4 (IgG4) positive fibroinflammatory response in meningeal biopsy. HP is a chronic inflammatory thickening of the dura mater which typically presents with headache, cranial nerve dysfunction and other neurological deficits. While first-line treatment with corticosteroids is recommended, many patients relapse and need additional immunosuppression. One recently described etiology is IgG4-related disease and in a subgroup of idiopathic patients, evidence suggests a crucial role of ANCA. To our knowledge, the simultaneous occurrence of IgG4-related disease and ANCA has not been reported so far. This man suffered life-threatening disease progression despite the administration of high dose steroids, cyclophosphamide and azathioprine. Treatment with rituximab was initiated which led to disappearance of clinical symptoms and decrease of dural thickening within weeks. This patient presents a possible disease overlap of IgG4-related and ANCA-associated HP and illustrates the effectiveness of rituximab in refractory IgG4-related HP.
authors: Department of Neurology, Ruhr University Bochum, University Hospital Knappschaftskrankenhaus Bochum, In der Schornau 23-25, Bochum 44892, Germany. Electronic address: popkirov@gmail.com.
authors: Stoyan
authors: S
authors: Popkirov
authors: Department of Neurology, Ruhr University Bochum, University Hospital Knappschaftskrankenhaus Bochum, In der Schornau 23-25, Bochum 44892, Germany.
authors: Thomas
authors: T
authors: Kowalski
authors: Department of Neurology, Ruhr University Bochum, University Hospital Knappschaftskrankenhaus Bochum, In der Schornau 23-25, Bochum 44892, Germany.
authors: Uwe
authors: U
authors: Schlegel
authors: Department of Neurology, Ruhr University Bochum, University Hospital Knappschaftskrankenhaus Bochum, In der Schornau 23-25, Bochum 44892, Germany.
authors: Sabine
authors: S
authors: Skodda
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000276:immunology*; D006801:Humans; D006984:Hypertrophy / Q000150:complications; D007074:Immunoglobulin G / Q000276:immunology*; D008297:Male; D008581:Meningitis / Q000188:drug therapy* / Q000276:immunology*; D008875:Middle Aged; D000069283:Rituximab / Q000627:therapeutic use*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007074:Immunoglobulin G; D000069283:Rituximab
keywords: Antineutrophil cytoplasmic antibodies; IgG4-related disease; Pachymeningitis; Rituximab