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Peripheral neuropathy associated with sicca complex

I A Grant1, G G Hunder, H A Homburger

  • 1Peripheral Neuropathy Research Center, Mayo Clinic, Rochester, MN 55905, USA.

Neurology
|April 1, 1997
PubMed
Summary
This summary is machine-generated.

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Peripheral neuropathy is a distinct syndrome in patients with sicca complex, often presenting before dry eye or dry mouth symptoms. Sensory neuropathies are most common, with systemic disease and vasculitis being rare.

Area of Science:

  • Neurology
  • Rheumatology
  • Immunology

Background:

  • Peripheral neuropathy is a known complication of Sjögren's syndrome, a systemic autoimmune disorder.
  • Neuropathy also occurs in isolated sicca complex (keratoconjunctivitis sicca and xerostomia), but its distinctness as a syndrome is unclear.

Purpose of the Study:

  • To retrospectively study patients with sicca complex and peripheral neuropathy.
  • To determine presentation modes, neuropathic patterns, serologic abnormalities, and systemic disease frequency.

Main Methods:

  • Retrospective study of 54 patients with sicca complex and peripheral neuropathy.
  • Analysis of presenting symptoms, neuropathic patterns, serologic markers (including antibodies to extractable nuclear antigens), minor salivary gland biopsy results, and clinical evidence of systemic disease.

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Main Results:

  • Peripheral neuropathy presented in 87% of patients; sicca symptoms were mild and often overlooked.
  • Sensory neuropathies predominated (61%), including sensory polyneuropathy and polyganglionopathy.
  • Minor salivary gland biopsy was positive in 73%; nonspecific inflammation was seen in 70% of nerve biopsies, but vasculitic neuropathy was rare.
  • Systemic disease and vasculitis were uncommon, particularly in the sensory polyganglionopathy group.
  • Antibodies to extractable nuclear antigens were present in only 10.4%.

Conclusions:

  • Peripheral neuropathy with isolated sicca complex constitutes a distinct syndrome where neuropathy is the primary feature.
  • Diagnosis requires assessment of ocular/salivary involvement and nerve biopsy findings.
  • Sensory polyneuropathy and polyganglionopathy are the predominant neuropathic patterns.
  • Serology is confirmatory but insensitive; systemic disease and vasculitis are less common than in typical Sjögren's syndrome.