Blau syndrome(BS) is characterized by a triad of granulomatous dermatitis, arthritis, and uveitis. Tumor necrosis factor inhibitors(TNFi) are treated for several refractory BS. Here, we report a case of Guillain-Barré syndrome (GBS) probably secondary to Yisaipu, a rare adverse drug reaction of TNFi in BS patients.

A 10-year-old girl was diagnosed with BS due to present with rash, arthritis, NOD2 gene mutation and biopsy 6 years ago, and treated by corticosteroid and methotrexate(MTX). However, she stopped all drugs by herself. Due to a recurrence, Yisaipu and MTX were given. After 9 months treatment, she developed pain, deviated mouth, weakness and absent knee jerk. CSF showed an albuminocytologic dissociation, positive anti-sulfatides IgM and electromyography showed peripheral nerve damage. Lumbar MRI showed enhancement signal of ventral roots, dorsal roots and cauda equina. 

The diagnosis of GBS was confirmed and she was initiated on corticosteroid(20mg/kg.d*3 days, then 2mg/kg.d). IVIG(2g/kg) and acyclovir were also given. She recovered fully from GBS one month later.

More attention should be paid to TNFi safety. The nervous system assessment should be recommended in follow-up.