Department of Neurology, Second Affiliated Hospital of Anhui Medical University, Hefei, China.
Department of Neurology, The Second People's Hospital of Hefei, Affiliated Hefei Hospital of Anhui Medical University, Hefei, China.
Front Immunol. 2023 Oct 5;14:1265609. doi: 10.3389/fimmu.2023.1265609. eCollection 2023.
Patients with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy can present with early neurological deterioration, but rapidly progressive respiratory failure is rarely reported. We present the cases of two patients with autoimmune GFAP astrocytopathy who experienced rapid progression to respiratory failure and were effectively treated using plasma exchange therapy.
Two patients were diagnosed with autoimmune GFAP astrocytopathy. Their initial symptoms were consistent with those of previously observed cases of autoimmune GFAP astrocytopathy. However, they experienced rapid progression to respiratory failure due to their lesion location. Specifically, case 1 had lesions in the medulla oblongata, and case 2 had lesions in the high cervical spinal cord, which are both common sites of lesions causing respiratory failure. The patients did not respond well to intravenous methylprednisolone and intravenous immunoglobulin initially and could not be withdrawn from ventilator support. Fortunately, subsequent plasma exchange therapy led to significant clinical improvements and successful withdrawal from ventilator support.
Patients with autoimmune GFAP astrocytopathy can present with rapidly progressive respiratory failure. Early treatment with plasma exchange can be beneficial in withdrawing patients from ventilator support.
患有自身免疫性神经胶质纤维酸性蛋白（GFAP）星形细胞瘤的患者可能会出现早期神经功能恶化，但很少有报告快速进展性呼吸衰竭的病例。我们报告了两例自身免疫性 GFAP 星形细胞瘤患者的病例，他们因病变部位而迅速进展为呼吸衰竭，并通过血浆置换治疗得到有效治疗。
两名患者被诊断为自身免疫性 GFAP 星形细胞瘤。他们的初始症状与之前观察到的自身免疫性 GFAP 星形细胞瘤病例一致。然而，由于病变位置，他们迅速进展为呼吸衰竭。具体来说，病例 1 的病变位于延髓，病例 2 的病变位于高颈段脊髓，这两个部位都是常见的导致呼吸衰竭的病变部位。患者最初对静脉注射甲基泼尼松龙和静脉注射免疫球蛋白反应不佳，无法脱离呼吸机支持。幸运的是，随后的血浆置换治疗导致了显著的临床改善，并成功地脱离了呼吸机支持。
患有自身免疫性 GFAP 星形细胞瘤的患者可能会出现快速进展性呼吸衰竭。早期采用血浆置换治疗可能有助于患者脱离呼吸机支持。
