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

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish (US)
Article number1174116
JournalFrontiers in Neurology
StatePublished - 2023

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

Cite this