Danieli Maria Giovanna, Pettinari Lucia, Marinangeli Lucia, Logullo Francesco
Scienze Cliniche e Molecolari, Clinica Medica, Torrette di Ancona, Italy.
BMJ Case Rep. 2012 Aug 8;2012:bcr0120125637. doi: 10.1136/bcr-01-2012-5637.
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.
急性脊髓炎是一种病因异质性的脊髓炎性疾病。我们报告了一名71岁复发性颈胸段脊髓炎女性患者,此次出现疾病新的复发。已排除视神经脊髓炎及其他急性和/或复发性脊髓病的可能病因。血清免疫球蛋白水平和特异性抗体反应符合常见可变免疫缺陷(CVID)的诊断。她接受了大剂量甲泼尼龙和静脉注射免疫球蛋白治疗。作为维持缓解药物,我们决定以高于CVID替代治疗通常使用剂量的0.2 g/kg/周皮下注射免疫球蛋白(CSL Behring)对她进行治疗。在3年随访中,治疗反应良好。未发生复发。我们的病例提示皮下注射免疫球蛋白在维持一名与CVID相关的复发性脊髓炎女性患者缓解及减少泼尼松用量方面的有效性和安全性。