Sjögren's syndrome in patients with chronic idiopathic axonal polyneuropathy

To assess the presence of symptoms and signs of Sjögren's syndrome in patients with otherwise idiopathic axonal polyneuropathy and to develop guidelines for the diagnostic approach with respect to Sjögren's syndrome in these patients.

Sixty five patients with axonal polyneuropathy in whom an aetiological diagnosis could not be made underwent (1) a standard interview focusing on ocular and oral sicca symptoms, (2) physical examination, (3) tests for objective assessment of keratoconjunctivitis sicca, (4) extensive serological investigations, and (5) a sublabial salivary gland biopsy.

In forty nine patients a sublabial salivary gland (SSG) biopsy was performed, thereby completing the whole investigation for Sjögren's syndrome. Three of these 49 patients (all women) had an SSG biopsy specimen suggestive of Sjögren's syndrome, which, in combination with other symptoms and signs, led to a diagnosis of primary Sjögren's syndrome.

None of the three patients with primary Sjögren's syndrome had spontaneously complained about sicca symptoms and the clinical neurological picture of them did not differ from the other patients in the study. Therefore, in patients with chronic idiopathic axonal polyneuropathy, especially in women, a systematic investigation for Sjögren's syndrome should be done, because the presence of Sjögren's syndrome may have implications for treatment and justifies a clinical follow up on a regular base.