Kidney involvement in idiopathic multicentric castleman disease-TAFRO syndrome: clinical spectrum, pathology, and outcomes

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ID: 328375
2026
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Abstract
Abstract Background Idiopathic multicentric Castleman disease -TAFRO syndrome (iMCD-TAFRO) is a clinically aggressive subtype. Renal involvement and its impact on prognosis are poorly characterized. We aimed to describe renal clinicopathological features and outcomes in a large iMCD-TAFRO cohort. Methods This 10-year retrospective cohort study enrolled iMCD-TAFRO patients at Peking Union Medical College Hospital. Renal involvement was defined by iMCD consensus criteria. Survival was analyzed using Kaplan-Meier methods. Results A total of 48 patients (70.8% male; mean age 45 ± 15 years) were included. Renal involvement was observed in 95.8% (46/48) of the patients, presenting predominantly with reduced urine output, mild hematuria and proteinuria, as well as elevated serum creatinine. Acute kidney injury (AKI) occurred in 80.4% (37/46) of patients showing renal involvement and 37.8% of them required renal replacement therapy (RRT). Renal biopsies (n = 7) revealed glomerular microangiopathy in six cases. AKI was associated with elevated C-reactive protein (CRP) and D-dimer levels, and CRP levels correlated with peak serum creatinine levels. Therapy targeting the iMCD was mainly bortezomib, cyclophosphamide and dexamethasone regimen (BCD) (31/48, 64.6%) with/without anti-IL-6 therapy. Nine patients died within hospital or during follow-up. Among AKI patients, 28/37 (75.7%) showed full recovery of renal function. Despite worse renal function at the baseline, no significant survival difference was observed between dialyzed and non-dialyzed patients. Conclusions Renal involvement in iMCD-TAFRO is common and frequently manifests as AKI associated with systemic inflammation. Early and aggressive therapy combined with supportive renal care, including timely dialysis, improved renal recovery and overall prognosis.
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Authors Shuo Zhang, Hanshu Liu, Siyuan Li, Yubing Wen, Wenling Ye, Wei Ye, Hua Zheng, Lubin Xu, Fei Teng, Ying Wang, Ke Zheng, Peng Xia, Lu Zhang, Li J, Xuemei Li, Limeng Chen
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Journal clinical kidney journal
Year 2026
DOI
10.1093/ckj/sfag326
URL
https://doi.org/10.1093/ckj/sfag326
Keywords Keywords not found

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