title: Unusual presentation Of Sjögren-associated neuropathy with plasma cell-rich infiltrate.
abstract: 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.
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
authors.forename: Elie
authors.initials: E
authors.lastname: Naddaf
authors.affiliation: Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
authors.forename: Sarah E
authors.initials: SE
authors.lastname: Berini
authors.affiliation: Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
authors.forename: P James
authors.initials: PJ
authors.lastname: B Dyck
authors.affiliation: Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
authors.forename: Ruple S
authors.initials: RS
authors.lastname: Laughlin
mesh_terms: D061605:Administration, Intravenous; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D015703:Antigens, CD / Q000378:metabolism; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000008:administration & dosage; D009460:Neurologic Examination; D020556:Neutrophil Infiltration / Q000502:physiology*; D010523:Peripheral Nervous System Diseases / Q000150:complications* / Q000000981:diagnostic imaging; D010950:Plasma Cells / Q000473:pathology*; D016111:Sjogren-Larsson Syndrome / Q000097:blood* / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D013497:Sural Nerve / Q000473:pathology / Q000648:ultrastructure
chemical_list: D000893:Anti-Inflammatory Agents; D015703:Antigens, CD; D008775:Methylprednisolone
keywords: GBS mimic; Sjögren syndrome; acute axonal neuropathy; acute sensory neuropathy; plasma cell infiltrate; sicca symptoms