{
  "abstract": "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.",
  "authors": [
    {
      "affiliations": [
        "Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"
      ],
      "name": "Tomohiro Suzuki"
    },
    {
      "affiliations": [
        "Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"
      ],
      "name": "Saeko Yamada"
    },
    {
      "affiliations": [
        "Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"
      ],
      "name": "Kanto Toriumi"
    },
    {
      "affiliations": [
        "Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"
      ],
      "name": "Masanori Kono"
    },
    {
      "affiliations": [
        "Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"
      ],
      "name": "Haruka Tsuchiya"
    },
    {
      "affiliations": [
        "Department of Allergy and Rheumatology, The University of Tokyo Hospital, Bunkyo, Tokyo, Japan"
      ],
      "name": "Keishi Fujio"
    }
  ],
  "title": "Potential stratification of TAFRO syndrome by anti-SS-A antibodies status and therapeutic response to rituximab",
  "uid": "7db01b8a-2353-59df-bca5-3b5bd0c518d6"
}