Kidney Week

Abstract: SA-PO248

A Case of Multicentric Castleman Disease Mimicking IgG4-Related Kidney Disease

Session Information

• Onconephrology: Immunological Cross-Talk
  November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Onconephrology

• 1700 Onconephrology

Authors

• Sawada, Erika, Department of Nephrology and Hypertension, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan

Erika Sawada,

• Shioda, Yuya, Department of Nephrology and Hypertension, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan

Yuya Shioda,

• Ono, Yuko, Department of Nephrology and Hypertension, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan

Yuko Ono,

• Hasegawa, Hajime, Department of Nephrology and Hypertension, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan

Hajime Hasegawa,

• Maeshima, Akito, Department of Nephrology and Hypertension, Saitama Medical Center, Saitama Medical University, Kawagoe, Saitama, Japan

Akito Maeshima,

Introduction

Multicentric Castleman disease (MCD) is a benign lymphoproliferative disorder with heterogenous clinical symptoms and involves systemic organs in addition to lymph nodes. Case reports have documented mesangial proliferative glomerulonephritis, membranoproliferative glomerulonephritis, and amyloidosis in patients with MCD. Here, we report a case of MCD showing numerous IgG4+plasma cell infiltration in the kidneys.

Case Description

A 55-year-old man was referred to our hospital for high fever and diarrhea. His laboratory data revealed anemia, renal dysfunction (eGFR 30 mL/min/1.73m²), polyclonal gammopathy (IgG 7130 mg/dL), an elevated serum IgG4 level (2130 mg/dL) and an elevated C-reactive protein level (8.0 mg/dL). Abdominal CT showed multiple lymph nodes swelling (axillary, hilar, para-aortic, and inguinal). Biopsy of the axillary lymph node showed expansion of the interfollicular areas by heavily infiltrating plasma cells. Renal biopsy showed a significant plasma cell infiltration into the tubulointerstitium. Immunohistochemical analysis revealed a 45% IgG4-positive:IgG-positive plasma cell ratio, which meets the diagnostic criteria for IgG4-related disease. Amyloid A deposition was found along the vessel walls. No abnormality was found in most glomeruli. IF showed no deposition of immunoglobulins and complements. Serological cytokine analysis revealed elevated levels of interleukin-6 (9.3 pg/ml)and vascular endothelial growth factor (VEGF) (1210 pg/ml). Human herpesvirus-8 was negative. Taken together with histological finding of lymph node biopsy, idiopathic MCD was diagnosed.

Discussion

Corticosteroid monotherapy resolved the serological and imaging abnormalities, but was partially effective. Additional therapy with tocilizumab is planned. Given that response to steroid therapy and prognosis are different between MCD and IgG4-related disease, diagnosis of MCD should be carefully made based on a combination of findings including organ distribution of disease, response to steroid therapy, and other pathological findings. Highly specific diagnostic biomarkers that can distinguish between these diseases are needed.