Kidney Week

Abstract: TH-PO550

Renal Injuries Induced by Castleman’s Disease

Session Information

• Trainee Case Reports - I
  October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

• 1202 Glomerular Diseases: Immunology and Inflammation

Authors

• Koshida, Takeo, Juntendo University Faculty of Medicine, Tokyo, Japan

Takeo Koshida,

• Suzuki, Hitoshi, Juntendo University Faculty of Medicine, Tokyo, Japan

Hitoshi Suzuki,

• Makita, Yuko, Juntendo University Faculty of Medicine, Tokyo, Japan

Yuko Makita,

• Gohda, Tomohito, Juntendo University Faculty of Medicine, Tokyo, Japan

Tomohito Gohda,

• Suzuki, Yusuke, Juntendo University Faculty of Medicine, Tokyo, Japan

Yusuke Suzuki,

Introduction

Castleman's disease is a benign lymphoproliferative disorder in which interleukin-6 (IL-6) is thought to play a pathogenetic role. Although renal involvement in Castleman's disease were reported, etiology of renal injuries is currently unknown, due to lack of data from renal biopsy. Here, we report two renal biopsied Castleman’s disease in which successfully treated with the tocilizumab, a humanized anti-IL-6 receptor antibody.

Case Description

< Case 1 >
A 30-year-old man presented swelling of systemic lymph node. Pathological analysis of cervical lymph node biopsy showed plasmacyte infiltration, and ruled out malignant lymphoma and IgG4-related disease. Laboratory data indicated anemic state, hypoalbuminemia, polyclonal hypergammaglobulinemia, high levels of CRP (9.1 mg/dl), IL-6 (20.6 pg/ml) and VEGF (513 pg/mL). Taken together, he was diagnosed as multicentric Castleman’s disease. The eGFR was 110.8 ml/min/1.73m², however, 0.6 g/day proteinuria and microscopic hematuria were detected. Then kidney biopsy was performed. The pathological findings of kidney biopsy specimen showed mild proliferation of mesangial cell. No immune deposits were detected by immunofluorescence analysis.
< Case 2 >
A 49-year-old man was detected anemia and swelling of systemic lymph node. Cervical lymph node was biopsied, and laboratory investigation showed anemic state, hypoalbuminemia, polyclonal hypergammaglobulinemia and high level of CRP (8.7 mg/dl), IL-6 (43.0 pg/ml) and VEGF (1250 pg/ml). Taken together, he was diagnosed as multicentric Castleman’s disease. The eGFR was 56.8 ml/min/1.73m², and 0.6g/day proteinuria and microscopic hematuria were detected at the time of renal biopsy. The pathological findings showed mild mesangial cell proliferation and global sclerosis in 10% of total glomerulus. Immunofluorescence analysis did not show any immune deposits.

Discussion

We present cases of kidney injury induced by Castleman's disease. In both cases, significant infiltration of plasmacyte and IL-6 positive cells were not detected in kidney biopsy specimens. Importantly, tocilizumab, but not corticosteroid successfully improved urinary abnormality. Although the mechanism and clinical course of renal involvement in Castleman’s disease is unclear, these cases suggested that kidney injury in patients of Castleman's disease is induced by systemic overproduced IL-6 which proliferate mesangial cells.