Plasma exchange for two patients with autoimmune GFAP astrocytopathy with rapid progression to respiratory failure: a case report

Frontiers in Immunology

Immunology

Yanghua Tian¹  Lan Xia¹  Jing Du¹  Shugang Cao²  Qi Li³ 

[1] Department of Neurology, Second Affiliated Hospital of Anhui Medical University, Hefei, China;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;Department of Neurology, The Second People’s Hospital of Hefei, Affiliated Hefei Hospital of Anhui Medical University, Hefei, China;

关键词: glial fibrillary acidic protein astrocytopathy;    respiratory failure;    plasma exchange;    intravenous methylprednisolone;    intravenous immunoglobulin;    efficacy;   

DOI  :  10.3389/fimmu.2023.1265609

 received in 2023-07-23, accepted in 2023-09-13,  发布年份 2023

来源: Frontiers

【 摘 要 】

BackgroundPatients 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.Case reportTwo 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did not respond well to intravenous methylprednisolone and intravenous immunoglobulin initially and could not be withdrawn from ventilator support.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.DiscussionPatients 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.

【 授权许可】

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Copyright © 2023 Du, Cao, Xia, Li and Tian