title: Recurrent myelitis in common variable immunodeficiency successfully managed with high-dose subcutaneous immunoglobulin.
abstract: Acute myelitis is an aetiologically heterogeneous inflammatory disorder of the spinal cord. We report on a 71-year-old woman with a recurrent cervical and thoracic myelitis who presented with a new relapse of the disease. Neuromyelitis optica was ruled out such as other possible causes of acute and/or recurrent myelopathy. Serum immunoglobulin levels and specific antibody responses were consistent with the diagnosis of common variable immunodeficiency (CVID). She was treated with high-dose methylprednisolone and intravenous immunoglobulin. As a remission-maintaining drug, we decided to treat her with subcutaneous immunoglobulin (CSL Behring) at 0.2 g/kg/week at doses higher than usually employed in replacement therapy in CVID. At 3-year follow-up, the response to treatment was good. No relapses occurred. Our case suggests the effectiveness and safety of subcutaneous immunoglobulin in maintaining remission and in sparing prednisone in a woman with recurrent myelitis associated with CVID.
authors.affiliation: Scienze Cliniche e Molecolari, Clinica Medica, Torrette di Ancona, Italy. m.g.danieli@univpm.it
authors.forename: Maria Giovanna
authors.initials: MG
authors.lastname: Danieli
authors.forename: Lucia
authors.initials: L
authors.lastname: Pettinari
authors.forename: Lucia
authors.initials: L
authors.lastname: Marinangeli
authors.forename: Francesco
authors.initials: F
authors.lastname: Logullo
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000097:blood; D017074:Common Variable Immunodeficiency / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000008:administration & dosage / Q000627:therapeutic use*; D007279:Injections, Subcutaneous; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000627:therapeutic use*; D009187:Myelitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D010146:Pain / Q000188:drug therapy; D011788:Quality of Life; D012008:Recurrence; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D007136:Immunoglobulins; D008775:Methylprednisolone