TAFRO syndrome complicating Sjögren's disease: response to subcutaneous tocilizumab

TAFRO syndrome is a rare hyperinflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly. Its coexistence with Sjögren's disease has only been reported in isolated cases, and both conditions are thought to share a pathogenic pathway mediated by the activation of the IL-6/VEGF axis and the presence of anti-SSA/Ro60 autoantibodies. We present a 33-year-old woman with primary Sjögren's disease who fulfilled the 2016 ACR/EULAR criteria and developed anasarca, persistent fever, severe thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy and tubulointerstitial nephritis. Despite receiving pulse therapy and high-dose glucocorticoids, she experienced progressive deterioration that required renal replacement therapy. Given the suspicion of TAFRO syndrome associated with a systemic inflammatory response, weekly subcutaneous tocilizumab was initiated, resulting in rapid improvement in her overall condition, resolution of anasarca, and complete recovery of renal function. This case highlights the rare overlap between TAFRO syndrome and Sjögren's disease, in which IL-6 activation plays a central role. The favorable response to IL-6 blockade emphasizes the importance of early recognition of this association and consideration of targeted therapies for refractory hyperinflammation.