Acute transverse myelopathy and cutaneous vasculopathy in primary Sjögren's syndrome.

Neurologic complications of primary Sjögren's syndrome (SS) may be under-estimated. Here, we report a patient with primary SS, who developed acute transverse myelopathy (ATM) and skin purpuric lesions simultaneously. In the first episode of myelopathy, the patient's neurologic deficits improved after steroid therapy. However, she died of recurrent myelopathy with systemic complications 4 months later. Review of the English-language literature revealed only 3 cases of primary SS associated with ATM, none of the 3 patients had skin lesions. Anti-Ro(SS-A) antibodies, and ATM in our patient suggests that immune-mediated vasculopathy may play a role in the pathogenesis of acute myelopathy in primary SS.