Neurological Signs in Sjogren’s Too Often Ignored


Clinicians should pay more attention to neurological signs in patients with high-activity primary Sjogren’s Syndrome or those with prior neurological involvements.

Clinicians should pay more attention to neurological signs in patients with high-activity primary Sjogren’s Syndrome or those with prior neurological involvements.

Among patients with primary Sjogren’s Syndrome (pSS), approximately 20 percent exhibit neurological manifestations. In many cases, neurological symptoms are the first characteristics to appear.

A study published this month in RMD Open divided neurological manifestations into both peripheral and central neurological manifestations. The new data breaks the overall 20-percent prevalence down, revealing that peripheral (PNS) and central neurological (CNS) manifestations occur in 15 percent and 5 percent of cases, respectively.

“These findings are consistent with previous reports indicating that about 20 percent of patients with pSS have neurological manifestations,” researchers wrote. “Nevertheless, few studies were as large as our study.”

Primary Sjogren’s Syndrome symptoms are pure sensory neuropathy, sensorimotor neuropathy, neuronopathy, cranial nerve involvement, polyneuropathy, mononeuritis multiplex and polyradiculoneuropathy. Central neurological manifestations are meningitis, meningoencephalitis, encephalitis, seizure, stroke, transverse myelitis, and cerebral vasculitis. Investigators also evaluated articular, cutaneous, muscular, renal, cardiovascular, and pulmonary involvement, Raynaud’s phenomenon, and vasculitis. [[{"type":"media","view_mode":"media_crop","fid":"48009","attributes":{"alt":"©AndreyPopov/","class":"media-image media-image-right","id":"media_crop_6839740077282","media_crop_h":"0","media_crop_image_style":"-1","media_crop_instance":"5709","media_crop_rotate":"0","media_crop_scale_h":"0","media_crop_scale_w":"0","media_crop_w":"0","media_crop_x":"0","media_crop_y":"0","style":"font-size: 13.008px; line-height: 1.538em; float: right;","title":"©AndreyPopov/","typeof":"foaf:Image"}}]]

According to investigators, the study had multiple objectives. First, researchers studied neurological manifestations prevalence in the French study, Assessment of Systemic Significant Evaluation in Sjögren’s Syndrome (ASSESS). Second, researchers investigated demographic and clinical characteristics of patients with PNS, CNS, or neurological manifestations, the influence of pre-existing neurological manifestations, and primary Sjögren’s Syndrome activity assessed using the EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI).

In a 15-center study, investigators examined neurological manifestations affecting the skin, joints, lungs, kidneys, PNS, CNS, muscles, and vessels of 392 individuals. Based on results, past or present neurological manifestations existed in 74 individuals (18.9 percent). PNS were present in 63 (16 percent), CNS in 14 (3.6 percent).

Pure sensory neuropathy and sensorimotor neuropathy were the most common primary Sjögren’s Syndrome symptoms, totaling 9.2 percent and 5.3 percent, respectively. Of the 14 patients with CNS manifestations, one had meningitis, five had seizures, five had cerebral vasculitis, and three had transverse myelitis.

Overall, new neurological manifestations were much more common among patients with prior neurological involvements (32.4 percent) than those without (6.7 percent).  Patients with and without neurological manifestations had 1.8 +/- 1.2 and 1.2 +/- 1.1 ESSDAI domain affected, respectively. The average ESSDAI in patients with and without neurological involvement was 9.4 +/- 6.8 and 4.3 +/- 4.8, respectively.



Guillermo Carvajal Alegria, Dewi Guellec, et al. "Epidemiology of neurological manifestations in Sjögren's syndrome: data from the French ASSESS Cohort," RMD Open 2016; 2:e000179 doi:10.1136/rmdopen-2015-000179

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