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 journalArticle

Abstract

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.

Original languageEnglish (US)
JournalMuscle and Nerve
DOIs
StateAccepted/In press - 2016

Fingerprint

Plasma Cells
Peripheral Nervous System Diseases
Sural Nerve
Methylprednisolone
Peripheral Nervous System
Arthralgia
Ataxia
Salivary Glands
Mouth
Fever
Lymphocytes
Biopsy
Therapeutics

Keywords

  • Acute axonal neuropathy
  • Acute sensory neuropathy
  • GBS mimic
  • Plasma cell infiltrate
  • Sicca symptoms
  • Sjögren syndrome

ASJC Scopus subject areas

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

Cite this

Unusual presentation Of Sjögren-associated neuropathy with plasma cell-rich infiltrate. / Naddaf, Elie; Berini, Sarah E.; Dyck, P. James B; Laughlin, Ruple S.

In: Muscle and Nerve, 2016.

Research output: Contribution to journalArticle

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abstract = "Introduction: Sj{\"o}gren 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 Sj{\"o}gren-associated, sensory predominant, length-dependent peripheral neuropathy mimicking Guillain-Barr{\'e} 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: Sj{\"o}gren syndrome can present with acute-onset, sensory predominant peripheral neuropathy. The role of plasma cells in Sj{\"o}gren syndrome is unexplored and deserves further study.",
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AU - Dyck, P. James B

AU - Laughlin, Ruple S.

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Y1 - 2016

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AB - 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.

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KW - Sicca symptoms

KW - Sjögren syndrome

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