Level of CSF CXCL10 is highly elevated and decreased after steroid therapy in patients with autoimmune glial fibrillary acidic protein astrocytopathy

Objectives

To examine the chemokine profile in the cerebrospinal fluid (CSF) of patients with glial fibrillary acidic protein astrocytopathy (GFAP-A), central nervous system immune-related adverse event (CNS-irAE), neurosarcoidosis (NS), neuromyelitis optica spectrum disorders (NMOSD), multiple sclerosis (MS), and human T-cell leukemia virus-1 (HTLV-1)-associated myelopathy (HAM).

Methods

The study included 38 patients presenting to St. Marianna University Hospital between May 2013 and November 2021 with GFAP-A, CNS-irAE, NMOSD, MS, NS, HAM and noninflammatory neurological diseases (NIND). We recorded the age, sex, duration of disease, brain/spinal lesions on magnetic resonance imaging (MRI), blood data, and measured chemokines (CXCL9, −10, −13, CCL3, −4, −17, −20, −22) in CSF. In patients with GFAP-A, clinical symptoms, and CSF CXCL10 levels were compared before and after steroid treatment.

Results

Patients with GFAP-A had higher CSF levels of CXCL10, CXCL13, and CCL22 (10736.1 [8786.7–149079.0] pg/ml (p < .05), 378.4 [239.9–412.2] pg/ml (p < .01) and 159.9 [130.5–413.9] pg/ml (p < .01), respectively). The CSF levels of CXCL10 improved from 10736.1 [8786.7–149079.0] pg/ml to 1879.0 [783.9–4360.0] pg/ml in patients with GFAP-A by steroid therapy.

Conclusion

CSF CXCL10 levels were particularly high in GFAP-A, and changes in levels after treatment correlated with clinical improvements, suggesting CXCL10 involvement in GFAP-A pathogenesis.