Tohoku J. Exp. Med., 2023 February, 259(4)

Rapid Clinical Improvement of Multicentric Castleman Disease (MCD) with Renal Involvement Following Treatment with Tocilizumab: AA Amyloidosis as a Possible Renal Involvement of MCD

Jumpei Temmoku,¹ Tomomi Sasajima,² Takeshi Kuroda,³ Yuya Sumichika,¹ Kenji Saito,¹ Shuhei Yoshida,¹ Haruki Matsumoto,¹ Yuya Fujita,¹ Naoki Matsuoka,¹ Tomoyuki Asano,¹ Shuzo Sato,¹ Toshiyuki Yamada,⁴ Yuko Hashimoto⁵ and Kiyoshi Migita¹

¹Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Fukushima, Japan
²Department of Rheumatology, Fukushima Rosai Hospital, Iwaki, Fukushima, Japan
³Department of Rheumatology and Nephrology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Niigata, Japan
⁴Department of Clinical Laboratory Medicine, Jichi Medical University, Shimotsuke, Tochigi, Japan
⁵Department of Pathology, Fukushima Medical University School of Medicine, Fukushima, Fukushima, Japan

Castleman disease (CD) is a lymphoproliferative disorder that manifests as hypergammaglobulinemia and severe inflammation with multiorgan involvement. However, renal involvement has been infrequently described in CD. We present a case of a 63-year-old Japanese male patient with multicentric CD (MCD) in whom kidney involvement, including impaired renal function and massive proteinuria, is present. He had a 2-year history of inflammatory arthritis and was referred to our clinic with newly developed proteinuria, renal dysfunction, and elevated levels of acute-phase proteins. Abdominal computed tomography scan revealed hepatosplenomegaly, including mesenteric and inguinal lymph node enlargements. The patient underwent inguinal lymph node resection. Excisional biopsy of the inguinal lymph node showed multiple lymphoid follicles and expansion of interfollicular areas by marked plasmacytosis consistent with mixed type CD. The patient was diagnosed with human herpes virus 8-negative MCD according to the international diagnostic criteria for CD. Diagnostic renal biopsy was not performed following the medical viewpoint. Tocilizumab (TCZ) treatment was highly effective in reducing proteinuria and stabilizing renal function, as well as improving other clinical symptoms. The patient responded to TCZ treatment, and the renal involvement was rapidly improved. Our preliminary immunohistochemical analysis indicated AA amyloid deposits in urinary epithelial cells suggesting a possible renal involvement of AA amyloidosis. TCZ could potentially be one of the therapeutic options in patients with MCD with renal involvement.

Keywords —— Castleman disease; renal involvement; tocilizumab

© 2023 Tohoku University Medical Press

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Tohoku J. Exp. Med 2023, 259, 285-291.

Correspondence: Kiyoshi Migita, M.D. Ph.D., Department of Rheumatology, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, Fukushima 960-1295, Japan.

e-mail: migita@fmu.ac.jp