ACUTE TRANSVERSE MYELOPATHY AND CUTANEOUS VASCULOPATHY IN PRIMARY SJOGRENS-SYNDROME

Neurologic complications of primary Sjogren's syndrome (SS) may be underestimated. 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 were present in 1 previously reported patient and in our patient. The coexistence of cutaneous vasculitis, 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.