Nerve ultrasound findings in Sjögren's syndrome‐associated neuropathy

Abstract

Background and Purpose:The phenotype of Sjögren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjögren's syndrome-associated neuropathy remains an underdiagnosed entity with only few insights considering the pathomechanisms of nerve damage. Nerve ultrasound has proven to be a useful and efficient tool in detecting nerve damage of autoimmune origin. We, therefore, aimed to evaluate this method for Sjögren's syndrome-associated neuropathy. Methods:Patients with Sjögren's syndrome and clinical signs of neuropathy underwent sonographic examination of both median and ulnar nerves. Nerve thickening was classified for cross-sectional areas of >12 mmš at the median nerve and for >10 mmš at the ulnar nerve. Fascicle thickening was documented for cross-sectional areas ≥5 mmš at the median and ≥3 mmš at the ulnar nerve.Results: Forty-three patients were included in the analysis (median age 60 years [interquartile range 53-73 years], female rate 60%). 31/43 patients (72%) showed abnormalities on nerve ultrasound, while nerve thickening was found more frequently than fascicle thickening (90% vs. 52% of patients with sonographic abnormalities, respectively). Abnormal findings were observed more frequently at the median nerve and in proximal localization. Abnormal findings on nerve conduction studies were evident in 36/43 patients (84%). Nerve conduction studies revealed a tendency of demyelinating nerve damage patterns being associated with abnormal findings on nerve ultrasound. Conclusions:In addition to nerve conduction studies, nerve ultrasound may have a supporting role in the diagnosis of Sjögren's syndrome-associated neuropathy. Also, our data support an immune-mediated inflammatory demyelinating pathogenesis of Sjögren's syndrome-associated neuropathy.

Saliva Free Light Chains in Patients with Neuro-Sjögren

“…These patients have been shown to have different clinical and paraclinical features compared with patients with SS without neurological involvement [ 3 , 25 ]. Patients with SS-associated polyneuropathy showed pathological results not only in nerve conduction, but also in ultrasound examinations of the nerves, and often meet the diagnostic criteria for CIDP [ 4 , 25 ]. However, compared with patients with CIDP and without SS, a higher frequency of women and cranial nerve affection has been reported [ 26 ].…”

“…Sjögren´s syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to sicca symptoms, but may also cause extra-glandular manifestations such as interstitial lung disease, arthritis, cutaneous vasculitis, and central or peripheral nervous system (CNS and PNS) involvement [ 1 , 2 , 3 , 4 , 5 , 6 , 7 ]. According to the latest American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria of 2016, SS can be diagnosed in a patient with sicca symptoms and additional anti-SSA(Ro)-antibody positivity and/or pathological focus scores on a minor salivary gland biopsy [ 8 , 9 , 10 ].…”

Trigeminal Nerve Affection in Patients with Neuro-Sjögren Detected by Corneal Confocal Microscopy

“…Considering the clinical score of the dry eye and the Ocular Surface Disease Index, there were no significant in-between-group differences between patients with Neuro-Sjögren and CIDP. Nevertheless, controls reached significantly better results at the clinical score of the dry eye when compared with the Neuro-Sjögren group (values are stated as median (interquartile range)): Neuro-Sjögren group 6 (4-8) (left) and 6 (4)(5)(6)(7)(8) [right]; CIDP group 4 (3-5) (left, p = 0.12) and 4 (3-6) (right, p = 0.12); controls 1 (0-3) (left, p = 0.003) and 2 (1-3) (right, p = 0.003).…”

“…Previous studies have found many similarities between the neuropathy associated with Sjögren’s syndrome and other autoimmune-mediated neuropathies such as chronic inflammatory demyelinating polyneuropathy. Both entities are associated with, for example, focal thickening of peripheral nerves and/or prominent fascicles [ 8 ]. In a comparison of CIDP patients with and without Sjögren’s syndrome, only cranial nerve impairment and female sex were more common in the subgroup with additional Sjögren’s syndrome, while clinical presentation as well as electrophysiological and laboratory findings of cerebrospinal fluid were similar [ 9 ].…”

Multimodal Assessment and Characterization of Sicca Syndrome

“…Given the inherent heterogeneity of such cohorts, such as the heterogeneous group of patients with pSS and varying manifestations, this finding is perhaps unsurprising ( 11 , 12 ). The aim of this study is to independently assess the role of SGUS in a large, unselected Sicca syndrome cohort that has undergone extended-criteria phenotypic clustering.…”