Primary Sjögren's syndrome patients with neuromyelitis optica spectrum disorders: clinical analysis of 4 cases and review of the literature

Abstract

Objective To explore the clinical manifestations, laboratory examination, MRI characteristics, treatment and prognosis of primary Sjögren's syndrome (PSS) with neuromyelitis optica spectrum disorders (NMOSDs). Methods The clinical manifestations, laboratory examination, MRI characteristics, treatment and prognosis of 4 patients diagnosed as PSS with NMOSDs were retrospectively analyzed. Results There were 2 PSS patients with neuromyelitis optica (NMO), 1 PSS patient combined with recurrent myelitis associated with longitudinally extensive spinal cord lesions (RLESLs), and 1 PSS patient with retrobulbar optic neuritis. Laboratory examination showed serum Sjögren's syndrome antigen A (SSA) antibody positive in 4 cases, Sjögren's syndrom antigen B (SSB) antibody positive in 3 cases and serum specific aquaporin 4 (AQP4) antibody positive in 3 patients with NMO. Abnormal features in cranial MRI mainly distributed in bilateral basal ganglia, cerebral peduncle corticospinal tract, beside lateral ventricles, and brainstem. Spinal MRI showed the lesions were mainly at cervical or thoracic spinal cord (≥ 3 vertebral segments) and also involved medullary bulb (2 cases). In acute stage, glucocorticoid and prednisone combined with immunosuppressive drugs were used. Conclusion PSS and NMO are autoimmune diseases, and the two diseases are closely linked. Patients with NMO and (or) NMOSDs were suggested to detect serum auto-antibody and AQP4 antibody. In patients with serum AQP4 antibody positive, the recurrence rate is high and prognosis is poor. Immunosuppressive therapy is recommended to reduce recurrence.