Sjögren’s disease complicated by TAFRO syndrome: a case report of fever and lymphadenopathy with literature review
Year: 2026
Type: article
Study design: Case Report
Abstract: TAFRO syndrome, a fulminant subtype of idiopathic multicentric Castleman disease (iMCD), is characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly. Its occurrence in patients with pre-existing Sjögren's disease (SjD) is rare and poses significant diagnostic and therapeutic challenges. A 59-year-old male with a 10-year history of SjD complicated by primary biliary cholangitis (PBC) presented with a 3-month history of recurrent epistaxis, fever, and lymphadenopathy. Laboratory findings revealed severe thrombocytopenia, markedly elevated inflammatory markers, and positive anti-SSA/Ro and AMA-M2 antibodies. PET-CT demonstrated generalized lymphadenopathy, splenomegaly, and bilateral pleural effusions. An extensive workup excluded infection, lymphoma, and hemophagocytic lymphohistiocytosis (HLH). Excisional lymph node biopsy confirmed Castleman disease (plasmacytic type), and integration of clinical and pathological findings established the diagnosis of TAFRO syndrome. The patient failed to respond to high-dose corticosteroids, intravenous immunoglobulin, and dexamethasone combined with ruxolitinib. Definitive disease control was ultimately achieved with the RPAD chemotherapy regimen (rituximab, bortezomib, doxorubicin, and dexamethasone), leading to sustained clinical remission with normalized platelet count at 12-month follow-up. This case highlights that TAFRO syndrome can arise on the background of chronic autoimmune disease as a life-threatening cytokine storm, and may be mistaken for refractory HLH. Early recognition through systematic exclusion of mimics and histological confirmation is essential. In patients who fail conventional and cytokine-directed therapies, intensive multi-agent chemotherapy such as the RPAD regimen may be an effective salvage option.
Source: Frontiers in Medicine
Institutions: Sir Run Run Shaw Hospital, Zhejiang University
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,801
OpenAlex ID: W7224192686