Unusual presentation Of Sjögren-associated neuropathy with plasma cell-rich infiltrate

Elie Naddaf, Sarah E. Berini, P. James B. Dyck, Ruple S. Laughlin

Research output: Contribution to journalArticlepeer-review


Introduction: Sjögren syndrome is thought to be a lymphocyte-driven process. Peripheral nervous system involvement occurs in about 20%–25% of patients. A sensory-predominant, large-fiber peripheral neuropathy is most common, and it is usually associated with a subacute to chronic presentation. Methods: We report a rare case of an acute Sjögren-associated, sensory predominant, length-dependent peripheral neuropathy mimicking Guillain–Barré syndrome. The patient presented with sensory ataxia preceded by fever and polyarthralgia. She gave a history of years of dry eyes and dry mouth. Results: She had a positive Shirmer test, abnormal salivary gland scan, and positive SS-A and SS-B antibodies. A sural nerve biopsy showed an unusual, dense, non-IgG4, polyclonal, plasma-cell perivascular infiltrate. The patient responded to treatment with weekly pulse intravenous methylprednisolone. Conclusions: Sjögren syndrome can present with acute-onset, sensory predominant peripheral neuropathy. The role of plasma cells in Sjögren syndrome is unexplored and deserves further study. Muscle Nerve 55: 605–608, 2017.

Original languageEnglish (US)
Pages (from-to)605-608
Number of pages4
JournalMuscle and Nerve
Issue number4
StatePublished - Apr 1 2017


  • GBS mimic
  • Sjögren syndrome
  • acute axonal neuropathy
  • acute sensory neuropathy
  • plasma cell infiltrate
  • sicca symptoms

ASJC Scopus subject areas

  • Clinical Neurology
  • Physiology (medical)
  • Cellular and Molecular Neuroscience
  • Physiology


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