# Case Report: Autoimmune glial fibrillary acidic protein astrocytopathy complicated with Sjogren’s syndrome and recurrent respiratory failure

**Authors:** Po-You Chen, Tzu-Han Lee, Wan-Lun Tsai, Hua-Ren Chang, Kuo-Sen Tseng, Cheng-Che Wu

PMC · DOI: 10.3389/fimmu.2025.1669415 · Frontiers in Immunology · 2025-10-01

## TL;DR

A man in his 50s with autoimmune GFAP-A and Sjogren’s syndrome showed improvement with steroids and rehabilitation, highlighting the disease's varied symptoms and treatment.

## Contribution

This case report adds to the understanding of GFAP-A's clinical spectrum and the potential role of rehabilitation in treatment.

## Key findings

- The patient showed clinical improvement with steroid treatment and rehabilitation training.
- MRI revealed longitudinal hypersignal from the anterior medulla to the C1 spinal cord.
- Autoimmune tests showed elevated GFAP and positive anti-Ro and anti-Smith antibodies.

## Abstract

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is a recently identified autoimmune encephalitis. We reported a case of a male in his 50s with autoimmune GFAP-A complicated with Sjogren’s syndrome and recurrent respiratory failure. The patient presented with acute and disabling encephalitis and myelitis, with symptoms including respiratory failure, swallowing dysfunction and limbs weakness. Autoimmune tests showed elevated GFAP and positive antinuclear antibody, anti-Ro and anti-Smith antibodies. MRI revealed longitudinal hypersignal from the anterior medulla to the C1 spinal cord. The clinical symptoms were favorably improved by steroid treatment and rehabilitation training. This case highlighted the spectrum of clinical manifestations associated with GFAP-A. Our findings also supported the effectiveness of rehabilitation training in treating this disease. Further investigation regarding diagnostic criteria, imaging characteristics, and the role of rehabilitation training in treating GFAP-A is necessary. This case was presented herein to shed more light on various aspects of this disease.

## Linked entities

- **Proteins:** GFAP (glial fibrillary acidic protein)
- **Diseases:** respiratory failure (MONDO:0021113)

## Full-text entities

- **Genes:** GFAP (glial fibrillary acidic protein) [NCBI Gene 2670] {aka ALXDRD}
- **Diseases:** limbs weakness (MESH:D018908), swallowing dysfunction (MESH:D003680), Sjogren's syndrome (MESH:D012859), myelitis (MESH:D009187), Autoimmune (MESH:D001327), autoimmune encephalitis (MESH:D020274), respiratory failure (MESH:D012131), encephalitis (MESH:D004660)
- **Chemicals:** steroid (MESH:D013256)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12521276/full.md

## References

23 references — full list in the complete paper: https://tomesphere.com/paper/PMC12521276/full.md

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Source: https://tomesphere.com/paper/PMC12521276