Sjögren's syndrome is an autoimmune disorder of unknown etiology. It occurs in isolation, known as primary Sjögren's syndrome, or secondary to a connective tissue disease. It primarily affects women over 50 years of age. Typical presentation involves dryness of the eyes, termed xeropthalmia, along with dryness of the mouth, termed xerostomia. The pathogenesis of the disorder involves a monocytic infiltration and damage to the salivary and lacrimal glands. Typical symptoms include dysphagia, pancreatitis, parotid enlargement, vasculitis, epistaxis, nasal crusting, corneal ulceration, dental caries, oral ulcers, and neurologic dysfunction. There is an increased risk of other malignancies associated with this disorder, particularly lymphoma.
Laboratory evaluation may demonstrate mild anemia, leukopenia, eosinophilia, elevated ESR, hypergammaglobulinemia, elevated rheumatic factor, SSA/Ro, or SSB/La. Diagnosis can be confirmed with a biopsy of the salivary glands, with the most typical site being a minor salivary gland within the lip. Schirmer's test can help with the diagnosis and is abnormal if less than 5 mm of the filter paper is moistened in five minutes when draped over the lower eyelid.
Neurologic involvement is observed in approximately 20% to 25% of patients with primary Sjögren's syndrome and can involve either the peripheral or the central nervous system (89). Focal neurologic deficits, diffuse neurologic dysfunction, and psychiatric as well as cognitive impairment have all been reported. Although episodes of neurologic impairment are typically transient, they often recur and are multifocal. Over time, patients typically sustain recurrent attacks and develop progressive neurologic impairment. Of those patients with neurologic involvement, the cranial nerves are involved in 19.5% of this subset. Cranial nerve involvements were distributed as follows: cochlear nerve (7.3%), trigeminal nerve (6.1%), facial nerve (4.9%), and olfactory nerve (2.5%) (90). Treatment is primarily supportive, but when neuropathy develops, several investigators have advocated cyclophosphamide therapy. Corticosteroid treatment may be less efficacious. Intravenous immunoglobulins and plasmapheresis have also been anecdotally reported to have benefit.
See Chapter 2 for detailed discussion of Sjogren's syndrome.
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