Vasculitic Neuropathy and Other Connective Tissue Disorders Associated with Neuropathy: Sjögren Syndrome

SJÖGREN SYNDROME

Sjögren syndrome (SS) is a systemic autoimmune disease that primarily affects middle-aged women. The criteria for diagnosis include sicca symptoms (dry eyes, dry mouth), objective evidence of keratoconjunctivitis, evidence of chronic lymphocytic sialoadenitis, and the presence of either anti–SS-A or anti–SS-B antibodies. Rash, arthralgias, and the Raynaud phenomenon are also common. Peripheral neuropathy is reported to occur in 10% to 30% of patients with SS. The neuropathy of SS is secondary to vasculitis in some cases and secondary to mononuclear cell infiltration without vasculitis (e.g., ganglionitis) in other cases. There may also be other mechanisms of neuropathy in these patients. For example, the etiology of the small fiber neuropathy seen in some cases of SS may be different. Several patterns of neuropathy are seen in association with SS: sensory ataxic neuropathy, painful sensory neuropathy without ataxia, multiple mononeuropathies, multiple cranial neuropathies, trigeminal sensory neuropathy, autonomic neuropathy with anhidrosis, and radiculoneuropathy. Abnormal pupils and orthostatic hypotension are relatively common accompaniments to many of these neuropathies and should be sought on examination.

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