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Abstract: SA-PO387

Two Cases of Multicentric Castleman Disease with Nephrotic Syndrome Treated with Tocilizumab

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Fukunaga, Naoya, Oita University, Yuhu, Japan
  • Aoki, Kohei, Oita University, Yuhu, Japan
  • Fukuda, Akihiro, Oita University, Yuhu, Japan
  • Nakata, Takeshi, Oita University, Yuhu, Japan
  • Uesugi, Noriko, Fukuoka university, Fukuoka-city,, FUKUOKA, Japan
  • Hisano, Satoshi, University of Occupational and Environment Health, Kitakyushu, Japan
  • Shibata, Hirotaka, Oita University Faculty of Medicine, Yufu city, Japan
Introduction

Multicentric Castleman disease (CD) is a systemic lymphoproliferative disease with an incompletely understood etiology. Renal complications of this disease have been reported in a few cases. We herein report two cases of multicentric CD with nephrotic syndrome treated with tocilizumab.

Case Description

Case 1
A 58-year-old man was diagnosed with multicentric CD by lymph node biopsy 7 years previously. He was followed closely without therapy because of his asymptomatic condition. He was admitted to our hospital with acute onset of nephrotic syndrome [albumin, 2.0 g/dl; urine protein:creatinine ratio (UP/UCr), 4 g/g creatinine). Light microscopic examination of kidney biopsy specimens revealed membranous nephropathy with cellular and fibrocellular crescents. Based on these findings, we diagnosed the patient with secondary membranous nephropathy due to CD. Steroid and tocilizumab therapy was started. After 3 months, the proteinuria had improved (UP/UCr < 1 g/g creatinine).
Case 2
A 49-year-old woman was diagnosed with multicentric CD by lymph node biopsy 9 years previously. She was followed closely without therapy because of her asymptomatic condition. She was admitted to our hospital with acute onset of nephrotic syndrome (albumin, 2.0 g/dl; UP/UCr, 5 g/g creatinine). Light microscopic examination of kidney biopsy specimens revealed Congo red stain-positive amyloid deposition. Based on these findings, we diagnosed the patient with secondary amyloidosis due to CD. Steroid and tocilizumab therapy was started.

Discussion

Few reports have described the various renal complications of CD, such as minimal change disease, mesangial proliferative glomerulonephritis, membranous glomerulonephritis, and amyloidosis. We have herein described two rare cases of multicentric CD with nephrotic syndrome and effective treatment with tocilizumab. Tocilizumab may be another therapeutic choice for multicentric CD with nephrotic syndrome.