General description

Sjögren syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration and inflammation of exocrine glands, particularly the lacrimal and salivary glands, leading to symptoms of dry eyes and dry mouth. It is most commonly seen in middle-aged and older women. A key feature of SS is its potential to cause extra-glandular manifestations, which affect various organs and systems, including the lungs, gastrointestinal tract, hematologic system, and nervous system.

Neurological complications are among the more severe extra-glandular features of SS and present with diverse manifestations such as neuropathy, myelopathy, myositis, psychiatric symptoms, and Parkinsonian syndromes. Longitudinally extensive myelitis is a known complication, and in some cases, SS may coexist with other autoimmune conditions, such as Neuromyelitis optica spectrum disorder (NMOSD), a relapsing autoimmune disease of the central nervous system.

Radiographic features

Peripheral neuropathy

Sjögren syndrome causes inflammation in the dorsal root ganglia, and if it extends to the posterior column, MRI shows T2WI hyperintensity.

Transverse myelitis

Sjögren syndrome is known to be associated with Neuromyelitis optica spectrum disorder (NMOSD), with spinal cord lesions often presenting as three or more long segments. Most of these lesions are AQP4-positive.

Cerebral lesion

In Sjögren syndrome, cerebral lesions are often associated with vasculitis, which can result in hyperintense signals on T2-weighted imaging in the cerebral white matter. Additionally, Sjögren syndrome may present with features similar to or overlapping with Neuromyelitis optica spectrum disorder (NMOSD). These include lesions in the dorsal medulla, around the third and fourth ventricles, the posterior limb of the internal capsule, and optic neuritis.