Neurologic involvement in seronegative primary Sjögren's syndrome with positive minor salivary gland biopsy: a single-center experience

Yoji Hoshina; Ka-Ho Wong; Jonathan Galli; Rae Bacharach; Julia Klein; Dorota Lebiedz-Odrobina; John W Rose; Bryan Trump; Christopher Hull; John E Greenlee; Stacey L Clardy

doi:10.3389/fneur.2023.1174116

Neurologic involvement in seronegative primary Sjögren's syndrome with positive minor salivary gland biopsy: a single-center experience

Front Neurol. 2023 Jun 9:14:1174116. doi: 10.3389/fneur.2023.1174116. eCollection 2023.

Authors

Yoji Hoshina¹, Ka-Ho Wong¹, Jonathan Galli^{1

2}, Rae Bacharach^{1

3}, Julia Klein¹, Dorota Lebiedz-Odrobina⁴, John W Rose^{1

2}, Bryan Trump⁵, Christopher Hull⁶, John E Greenlee^{1

2}, Stacey L Clardy^{1

2}

Affiliations

¹ Department of Neurology, University of Utah, Salt Lake City, UT, United States.
² George E. Wahlen Veterans Affairs Medical Center, Salt Lake City, UT, United States.
³ Department of Neurology, Penn State Health, Hershey, PA, United States.
⁴ Department of Medicine, Division of Rheumatology, University of Utah, Salt Lake City, UT, United States.
⁵ School of Dentistry, University of Utah, Salt Lake City, UT, United States.
⁶ Department of Dermatology, University of Utah, Salt Lake City, UT, United States.

Abstract

Objective: To assess the demographics, neurologic manifestations, comorbidities, and treatment of patients with seronegative primary Sjögren's syndrome (pSS).

Patients and methods: We conducted a retrospective chart review on patients with seronegative pSS evaluated by a neurologist at the University of Utah Health between January 2010 and October 2018. The diagnosis was based on characteristic symptoms, positive minor salivary gland biopsy according to the American-European Consensus Group 2002 criteria, and seronegative antibody status.

Results: Of 45 patients who met the study criteria, 42 (93.3%) were Caucasian, and 38 (84.4%) were female. The patients' mean age at diagnosis was 47.8 ± 12.6 (range 13-71) years. Paresthesia, numbness and dizziness, and headache were noted in 40 (88.9%), 39 (86.7%), and 36 patients (80.0%), respectively. Thirty-four patients underwent brain magnetic resonance imaging. Of these, 18 (52.9%) showed scattered nonspecific periventricular and subcortical cerebral white matter T2/fluid-attenuated inversion recovery hyperintense foci. Twenty-nine patients (64.4%) presented to the neurology clinic prior to pSS diagnosis, and the median delay in diagnosis from the first neurology clinic visit was 5 (interquartile ranges 2.0-20.5) months. Migraine and depression were the most common comorbidities in 31 patients (68.9%). Thirty-six patients received at least one immunotherapy, and 39 were on at least one medication for neuropathic pain.

Conclusion: Patients often display various nonspecific neurological symptoms. Clinicians should express a high degree of skepticism regarding seronegative pSS and consider minor salivary gland biopsy to avoid delaying diagnosis, as undertreatment can affect patients' quality of life.

Keywords: lip biopsy; neuropathic pain; nonspecific neurological symptoms; primary Sjögren syndrome (pSS); seronegative.