Potential stratification of TAFRO syndrome by anti-SS-A antibodies status and therapeutic response to rituximab.

OBJECTIVES

Thrombocytopaenia, anasarca, fever, reticulin fibrosis, renal dysfunction and organomegaly (TAFRO) syndrome has been reported to be associated with anti-SS-A antibodies, which activate type I interferon (IFN) signalling. We propose a subclassification of TAFRO syndrome based on the anti-SS-A antibody status, with the aim of identifying effective treatment strategies.

METHODS

We reviewed TAFRO syndrome cases from the PubMed/MEDLINE databases from January 1977 to October 2024. The information on concomitant autoimmune diseases, the presence of anti-SS-A antibodies, serum IgG levels, treatment courses and clinical outcomes was collected and retrospectively analysed. Patients were stratified according to anti-SS-A antibody status, and response to tocilizumab (TCZ) or rituximab (RTX) was evaluated.

RESULTS

Among 212 cases, 27 had concomitant autoimmune diseases, with Sjögren's disease (SjD) being the most common. Anti-SS-A antibody positivity was observed in 37 cases, of which 12 fulfilled the classification criteria for SjD. RTX was significantly more effective in anti-SS-A antibody-positive patients than in antibody-negative patients (88.9% vs 16.7%, p=0.011), whereas therapeutic response to TCZ was not affected by anti-SS-A antibody status. Serum IgG levels were significantly higher in anti-SS-A antibody-positive patients than in antibody-negative patients (p=0.020).

CONCLUSIONS

TAFRO syndrome shows clinical heterogeneity and anti-SS-A antibody status may represent one potential factor associated with treatment response. Further studies are needed to confirm these findings and clarify the underlying mechanisms.