Abstract: FR-PO1273
Castleman Disease with Renal Involvement: Case Series
Session Information
- Onconephrology: Diagnostic Dilemmas, Therapy-Related Toxicities, and Clinical Cases
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onconephrology
- 1600 Onconephrology
Authors
- Almalki, Lamis A., Prince Sultan Military Medical City, Riyadh, Riyadh Province, Saudi Arabia
- Weber, Nina Teresa, Stadtspital Zurich Triemli, Zürich, ZH, Switzerland
- Kitchlu, Abhijat, University Health Network, Toronto, Ontario, Canada
- Vohra, Harjot Singh, University Health Network, Toronto, Ontario, Canada
- John, Rohan, University Health Network, Toronto, Ontario, Canada
Introduction
Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder driven by cytokine dysregulation, particularly interleukin-6 (IL-6). Renal involvement is uncommon but increasingly recognized, with diverse histopathological patterns. We describe a case series highlighting the spectrum of kidney manifestations and clinical outcomes in biopsy-proven iMCD-associated renal disease
Case Description
Four patients with iMCD (including one TAFRO subtype) presented with variable renal involvement. Case 1 demonstrated mild renal impairment with podocyte injury and stable CKD stage 3b following rituximab-based therapy. Case 2 presented with immune-complex glomerulonephritis and nephrotic-range proteinuria, had improvement after transition to anti–IL-6 therapy. Case 3 exhibited progressive renal disease evolving from chronic tubulointerstitial injury to focal segmental glomerulosclerosis, despite systemic disease control, ultimately progressing to CKD stage 3b–4. Case 4, a TAFRO phenotype, presented with thrombotic microangiopathy and acute kidney injury, achieving complete renal recovery after prompt corticosteroids and siltuximab.
Discussion
Renal manifestations in iMCD are heterogeneous, ranging from reversible inflammatory lesions to irreversible fibrotic damage. IL-6–mediated endothelial injury, immune dysregulation, and cytokine-driven inflammation play central roles in pathogenesis. Anti–IL-6 therapy remains the cornerstone of treatment, with favorable outcomes particularly in early or inflammatory lesions. However, progression to chronic kidney disease may occur despite systemic remission, emphasizing the importance of early recognition and kidney biopsy. Histopathology appears to be a key determinant of prognosis, with inflammatory patterns showing reversibility, while chronic fibrosis predicts poorer outcomes.