A Case of Repeated TAFRO Syndrome-Like Symptoms and Retroperitoneal Hemorrhage in a Patient With Sjögren Syndrome

A 50-year-old Japanese man complaining of dry mouth and eyes, pale skin with cold irritation, and worsening epigastric pain was admitted to the hospital, whereupon he developed fever and anasarca. A computed tomography (CT) scan showed ascites, hepatosplenomegaly, and mildly enlarged multiple lymph nodes, and blood examination revealed renal impairment, thrombocytopenia, and high levels of C-reactive protein (CRP). He was diagnosed with Sjögren syndrome and concurrently manifested symptoms resembling TAFRO syndrome (i.e., thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O)). Although the TAFRO syndrome-like symptoms progressed, he gradually recovered with immunosuppressive agents. Seven years and five months after the admission, the TAFRO syndrome-like symptoms recurred. Bone marrow biopsy specimens revealed reticulin fibrosis. Inguinal and mediastinal lymph nodes biopsy specimens revealed Castleman disease-like features. Although the symptoms indicated TAFRO syndrome, a diagnosis was not possible owing to the presence of hypergammaglobulinemia and Sjögren syndrome, which required exclusion. Corticosteroid treatment was initiated; however, it was complicated by retroperitoneal hemorrhage, probably due to microangiopathy. After additional treatment with tocilizumab and rituximab, the TAFRO syndrome-like symptoms improved and the hemorrhage progression stopped. In conclusion, TAFRO syndrome-like symptoms may recur with vascular complications and can be successfully treated with tocilizumab and rituximab during Sjögren syndrome. The etiology of TAFRO syndrome could potentially involve Sjögren syndrome, and these syndromes may co-exist.