Abstract: TH-PO1044
Clinical Course and Recurrence in TAFRO Syndrome: A Case Series Analysis
Session Information
- CKD Progression and Complications: Diagnosis, Prognosis, Risk Factors
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: CKD (Non-Dialysis)
- 2302 CKD (Non-Dialysis): Clinical, Outcomes, and Trials
Authors
- Yoshimura, Yusuke, Toranomon Byoin Bunin, Kawasaki, Kanagawa, Japan
- Mizuno, Hiroki, Toranomon Byoin Bunin, Kawasaki, Kanagawa, Japan
- Sawa, Naoki, Toranomon Byoin Bunin, Kawasaki, Kanagawa, Japan
Background
TAFRO syndrome is a rare systemic inflammatory disease characterized by five manifestations: thrombocytopenia, anasarca, fever/inflammation, renal insufficiency/reticulin fibrosis of the bone marrow, and organomegaly. It is considered a severe subtype of idiopathic multicentric Castleman disease (iMCD), with the potential for dire outcomes such as hemodialysis or death. Despite prior reporting on TAFRO syndrome's clinicopathological features, its long-term clinical course remains unknown. This study presents a case-series analysis of ten patients with TAFRO syndrome, exploring the long-term prognosis and recurrence of TAFRO syndrome.
Methods
We conducted a retrospective case-series study of patients with TAFRO syndrome at Toranomon Hospital or Toranomon Hospital Kajigaya from January 2012 to June 2021. Diagnosis was based on the criteria proposed by Nishimura et al. We collected clinicopathological data from medical records. Clinical course analysis was performed, with the diagnosis day defined as Day 1.
Results
A total of 10 patients were enrolled. All patients were Japanese, with a median follow-up period of 1,636 days (185 – 3,566). All patients underwent corticosteroid treatment, with 9 out of 10 receiving tocilizumab (TCZ) therapy. Rituximab and cyclosporin A were added in 4 and 1 patients, respectively. Recurrence occurred in 2 cases after reducing the TCZ dosage. By contrast, 2 patients maintained long-term remission after terminating all medications. One patient died due to severe pneumonia during immunosuppressive therapy.
Conclusion
The study revealed the potential for disease recurrence in TAFRO syndrome, particularly following TCZ dosage reduction. These findings suggest that the diathesis of TAFRO syndrome may persist long after the initial onset of the disease, and insufficient maintenance therapy could trigger a relapse. On the other hand, some patients maintained long-term remission even after stopping TCZ, indicating the possible varying degrees of IL-6 contribution to the etiology of TAFRO syndrome. Further studies are needed to enhance our understanding of this rare disease's pathogenesis and to optimize its stratified management.