Short report
Sjögren's syndrome in patients with chronic idiopathic axonal
polyneuropathy
a Departments of Neurology, b Rheumatology and Clinical Immunology, University Hospital,
Utrecht, The Netherlands, c Department of Neurology, St Lucas-Andreas Hospital, Amsterdam,
The Netherlands
Correspondence to: Dr GW van Dijk, Department of Neuromuscular Diseases, University Hospital, Utrecht, PO Box 85500, C03.236, 3508 GA Utrecht, The Netherlands.
Received 21 January 1997 and in revised form 19 March 1997;
Accepted 3 April 1997
OBJECTIVE
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.
METHODSSixty 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.
RESULTSIn 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.
CONCLUSIONSNone 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.
© 1997 by Journal of Neurology, Neurosurgery, and Psychiatry
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