Sjogrens Syndrome Complicated with Optic Neuromyelitis and Dual Positivity of AQP4 and GFAP Antibodies: A Case Report

A patient diagnosed with Sjogren's syndrome (SS) developed weakness and numbness in both lower limbs, accompanied by abdominal distension and sensory impairment in the left index and little fingers.Cervical magnetic resonance imaging (MRI) indicated the presence of longitudinal inflammatory lesions in the C3-C6 segments of the cervical spinal cord.The serum tests showed positive antiaquaporin-4 (AQP4) and anti-glial fibrillary acidic protein (GFAP) antibodies.After treatment with steroid pulse and immunosuppressive agents, the symptoms of limb numbness, abdominal distension, and sensory impairment in the left finger were significantly improved.Cervical spinal cord MRI indicated significant improvement in inflammatory lesions.Anti-GFAP antibodies are quite uncommon in cases of SS complicated with neuromyelitis optica, and it is necessary to identify the true autoimmune GFAP-astrocytopathy (GFAP-A), if it is positive.

Publication Details

Journal
Journal of College of Physicians And Surgeons Pakistan
Published
2025-01-01
DOI
https://doi.org/10.29271/jcpspcr.2025.212
Primary Topic
Salivary Gland Disorders and Functions
Type
article
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article

Sjogrens Syndrome Complicated with Optic Neuromyelitis and Dual Positivity of AQP4 and GFAP Antibodies: A Case Report

Journal of College of Physicians And Surgeons Pakistan
Salivary Gland Disorders and Functions
article

Sjogrens Syndrome Complicated with Optic Neuromyelitis and Dual Positivity of AQP4 and GFAP Antibodies: A Case Report

article en

Abstract

A patient diagnosed with Sjogren's syndrome (SS) developed weakness and numbness in both lower limbs, accompanied by abdominal distension and sensory impairment in the left index and little fingers.Cervical magnetic resonance imaging (MRI) indicated the presence of longitudinal inflammatory lesions in the C3-C6 segments of the cervical spinal cord.The serum tests showed positive antiaquaporin-4 (AQP4) and anti-glial fibrillary acidic protein (GFAP) antibodies.After treatment with steroid pulse and immunosuppressive agents, the symptoms of limb numbness, abdominal distension, and sensory impairment in the left finger were significantly improved.Cervical spinal cord MRI indicated significant improvement in inflammatory lesions.Anti-GFAP antibodies are quite uncommon in cases of SS complicated with neuromyelitis optica, and it is necessary to identify the true autoimmune GFAP-astrocytopathy (GFAP-A), if it is positive.

Journal of College of Physicians And Surgeons PakistanVol. 35(01)
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Salivary Gland Disorders and Functions
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