Refractory Sjögren's syndrome myelopathy successfully treated with subcutaneous tocilizumab: A case report

RATIONALE: It is known that 5% to 34% of Sjögren's syndrome (SS) cases are complicated by neuropathy in the form of myelitis. Although SS myelopathy (SSM) is often treated with glucocorticoid (GC) and immunosuppressants such as cyclophosphamide (CY), a therapeutic strategy for SSM has not been established. PATIENT CONCERNS: A 65-year-old female was admitted with weakness and thermal hypoalgesia in the lower limbs. Four months before this admission, she showed weakness in her lower limbs and thermal hypoalgesia of bilateral upper and lower limbs. Magnetic resonance imaging (MRI) revealed that the cause of her neurological symptoms was cervical myelitis. She was diagnosed with SS because she tested positive for the ophthalmic test (Schirmer's test and fluorescent test) and for the anti-SS-A antibodies. Therefore, myelitis was thought to be a complication of SS. She was treated with GC and CY. Both neurological symptoms and MRI findings temporarily improved, and the GC dose was gradually decreased. One month before this admission, her neurological symptoms and MRI findings were exacerbated. Upon relapse of SSM, serum amyloid A protein (SAA) level was markedly elevated. DIAGNOSES: Based on MRI findings, the diagnosis was SSM relapse. INTERVENTIONS: Treatment by subcutaneous tocilizumab (TCZ) 162 mg every two weeks was introduced. OUTCOMES: After introducing TCZ, her neurological symptoms and MRI findings gradually improved. SAA levels remained low. At eight months after the introduction of TCZ, the GC dose has been decreased and so far, the myelitis has not relapsed. LESSONS: This case report is the first report suggesting the effectiveness of TCZ for refractory SSM. Subcutaneous TCZ might be an effective therapeutic option for treating refractory SSM when SAA levels are elevated.