Autoinflammatory & rare systemic diseases

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

RMD OpenPublished Systematic review of published cases

Authors' conclusion

TAFRO syndrome shows clinical heterogeneity and anti-SS-A antibody status may represent one potential factor associated with treatment response.

Study context

Population
Published cases of TAFRO syndrome, PubMed/MEDLINE, January 1977 to October 2024
Participants
212 published cases
Comparison
Anti-SS-A antibody-positive vs antibody-negative TAFRO syndrome
  • Anti-SS-A positiven = 37
Outcome
Response to rituximab (RTX) or TCZ by anti-SS-A antibody status
Methodological detailsretrospective analysis, literature review (PubMed/MEDLINE, January 1977 to October 2024)
  • Retrospective analysis
  • Literature review (PubMed/MEDLINE, January 1977 to October 2024)

Pooled result

As reported in the abstract

Response to RTX

P = 0.011

Further pooled results

OutcomeP value
Serum IgG levelsSignificantly higher in anti-SS-A positiveP = 0.020

Response to tocilizumab (TCZ)

Therapeutic response to TCZ not affected by anti-SS-A antibody status

Concomitant autoimmune diseases

Cases with concomitant autoimmune diseases (Sjögren's disease (SjD) most common)
27
Anti-SS-A positive cases fulfilling SjD classification criteria
12

Original publication

Potential stratification of TAFRO syndrome by anti-SS-A antibodies status and therapeutic response to rituximab. RMD Open. Published 21 May 2026. doi:10.1136/rmdopen-2025-006547

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