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Peripheral neuropathy in primary Sjögren's syndrome
S I Mellgren1, D L Conn, J C Stevens
1Peripheral Neuropathy Research Laboratory, Mayo Clinic, Rochester, MN 55905.
Neurology
|March 1, 1989
Summary
Sjögren's syndrome can cause peripheral neuropathy, often presenting as sensorimotor or sensory polyneuropathy. Necrotizing vasculitis is a key factor in nerve fiber damage, suggesting this condition should be considered in unexplained neuropathies.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Sjögren's syndrome is an autoimmune disorder primarily affecting exocrine glands, leading to dryness of mucous membranes.
- It can also manifest with systemic complications, including neurological involvement affecting the central and peripheral nervous systems.
Purpose of the Study:
- To investigate the clinical and pathological features of peripheral neuropathy in patients with primary Sjögren's syndrome.
- To identify potential underlying mechanisms and guide diagnosis in cases of unexplained neuropathy.
Main Methods:
- Retrospective review of 33 patients with primary Sjögren's syndrome and peripheral neuropathy.
- Neurologic examinations, electromyography (EMG), and sural nerve biopsy in 11 patients.
Main Results:
- Symmetric sensorimotor polyneuropathy was the most common presentation, followed by sensory neuropathy.
- Autonomic, mononeuropathy, or cranial neuropathies occurred in about 25% of patients.
- Nerve biopsies revealed necrotizing vasculitis and axonal degeneration as primary pathologies.
Conclusions:
- Peripheral neuropathy is a significant neurological manifestation of Sjögren's syndrome.
- Necrotizing vasculitis is implicated in nerve damage, and Sjögren's syndrome should be considered in patients with sensory, autonomic, or trigeminal neuropathy of unknown cause.