Successfully treated refractory Sjögren’s syndrome myelopathy with tocilizumab; case report

Introduction and importance: myelitis in patients with Sjögren’s syndrome is rare and its incidence does not exceed 5%. It is usually treated with corticosteroids, immunosuppressants, and different treatment lines that have not yet been agreed upon. Case presentation: A 59-year-old man with a 10-year history of primary Sjögren’s syndrome (PSs) based on the presence of dry mouth and eyes, Schirmer’s test positivity, and anti-SS-A positivity. Suffering from about 3 months of history of weakness in the upper and lower extremities, about 3 months ago, Magnetic resonance imaging (MRI) consisting of cervical myelitis was performed. Because there was no other cause due to his previous Sjögren’s condition, Sjögren’s myelitis was considered a complication of PSs. He was placed on a treatment plan that included cyclophosphamide and corticosteroids, with good improvement clinically and radiologically on the MRI. Accordingly, he began gradually reducing the cortisone, but unfortunately, the patient relapsed and the symptoms worsened significantly, both clinically and on the MRI, before the patient entered the hospital. An increase in serum amyloid A protein (SAA) level was also found. Here, the treatment was moved to biological treatment. The patient was given subcutaneous tocilizumab (TCZ), 162 mg every 2 weeks, and the gradual improvement was good. No relapses occurred, and the level of SAA remained low until 8 months from the start of treatment. Corticosteroids were started to be reduced, and no relapses occurred in clinically and radiological symptoms. Discussion: We mentioned in this manuscript the case of a patient with PSs who had a complication of a neurological injury, namely cervical myelitis. He improved significantly on treatment with subcutaneous TCZ every 2 weeks, with complete remission of clinical and radiological symptoms and a decrease in the serum level of SAA, even after reducing the dose of cortisone without the risk of serious adverse events such as infections. Conclusion: In this case, subcutaneous TCZ has proven effective in treating myelitis when high protein amyloid A (SAA) levels are present in the blood. This may be the first case describing the effectiveness of TCZ in treating this condition.