Case Report

Primary Sjögren’s Syndrome Accompanied by Clinical Features of TAFRO Syndrome

Table 2

Comparison summary of previous cases of Sjögren’s syndrome complicated with clinicopathologic features of TAFRO syndrome.

AuthorsIwanaga et al.Fujimoto et al.Takasawa et al.Li et al.Kojima et al.Our case

Sjögren’s syndromeHistopathology++NDNDNDND
Oral examination+ScintigraphyNDNDND+
Ocular examination+++NDND
Anti-Ro/SS-A antibody++++++
Anti-La/SS-B antibody+ND++
TAFRO syndromeMajor categoriesAnasarca++++++
Thrombocytopenia++++++
Systemic inflammation++++++
Minor categoriesHistology of lymph node biopsy++++ND
Reticulin myelofibrosis++++ND
Mild organomegaly++++++
Renal insufficiency+++ND±
TreatmentsPSL, CyAPSLPSL, CyASiltuximab, PSL, RTX, CY, vincristinePSLPSL, CyA, eltrombopag

CY, cyclophosphamide; CyA, cyclosporine; PSL, prednisolone; RTX, rituximab; ND, not determined.