Combined B-cell immunomodulation with rituximab and belimumab in severe, refractory TAFRO syndrome associated with Sjögren’s syndrome: A case report

ABSTRACT TAFRO syndrome is a systemic inflammatory disease of unknown aetiology. It is characterised by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. Herein, we report the case of a 60-year-old male with TAFRO syndrome. A few weeks after the patient developed an intermittent fever, he presented to our hospital with diarrhoea, abdominal distension, and whole-body oedema (face, extremities, and abdomen). Autoantibody and lip biopsy findings supported the diagnosis of primary Sjögren’s syndrome. High-dose steroids and tocilizumab were used to treat his refractory thrombocytopenia and ascites. However, systemic inflammation and renal dysfunction did not improve, resulting in temporary haemodialysis. Eventually, combined B-cell immunomodulation therapy with rituximab and belimumab ameliorated the patient’s symptoms. About 16 weeks after discharge, the overall condition of the patient had improved. The TAFRO syndrome may be a severe manifestation of primary Sjögren’s syndrome. Considering the immunological context, combined B-cell immunomodulation therapy provides new insights into improving this life-threatening disease and enables rapid steroid tapering.