TY - JOUR
T1 - Case report
T2 - Importance of early and continuous tocilizumab therapy in nephrotic syndrome associated with idiopathic multicentric Castleman disease: A case series
AU - Kojima, Daiki
AU - Yamaguchi, Shintaro
AU - Hashiguchi, Akinori
AU - Hayashi, Kaori
AU - Uchiyama, Kiyotaka
AU - Yoshimoto, Norifumi
AU - Adachi, Keika
AU - Nakayama, Takashin
AU - Nishioka, Ken
AU - Tajima, Takaya
AU - Morimoto, Kohkichi
AU - Yoshino, Jun
AU - Yoshida, Tadashi
AU - Monkawa, Toshiaki
AU - Kanda, Takeshi
AU - Itoh, Hiroshi
N1 - Funding Information:
We would like to thank the treating medical staff for their skillful care of these patients and Editage (www.editage.com) for English language editing.
Publisher Copyright:
Copyright © 2023 Kojima, Yamaguchi, Hashiguchi, Hayashi, Uchiyama, Yoshimoto, Adachi, Nakayama, Nishioka, Tajima, Morimoto, Yoshino, Yoshida, Monkawa, Kanda and Itoh.
PY - 2023/1/9
Y1 - 2023/1/9
N2 - Idiopathic multicentric Castleman disease (iMCD) is a systemic and polyclonal lymphoproliferative disease involving multiple organs, including the kidneys, due to the overproduction of interleukin-6 (IL-6). Recently, several reports have suggested that excessive IL-6 actions in iMCD could have a causal relationship with the development of diverse histopathological renal manifestations that cause nephrotic syndrome. However, the treatment for such cases remains unclear. We report a series of three cases of nephrotic syndrome due to iMCD that helps to delineate the importance of early and continuous therapy with the anti-interleukin-6 receptor antibody tocilizumab. First, treatment was suspended for infectious control, and the patient presented with nephrotic syndrome due to diffuse mesangial and endocapillary hypercellularity without immune deposits complicating acute kidney injury. Second, iMCD was treated with prednisolone alone. The patient suddenly developed nephrotic syndrome due to immune-complex glomerulonephritis, not otherwise specified, complicated with acute kidney injury. In the third case, nephrotic syndrome secondary to membranous glomerulonephritis was diagnosed, with a skin rash and IgE antibodies to tocilizumab, and was therefore treated with prednisolone alone. In contrast to the first two cases, the third progressed to end-stage renal disease on hemodialysis. Taken together, this series suggests that clinicians should maintain clinical vigilance for iMCD as a possible underlying component of nephrotic syndrome, since iMCD presents with a variety of renal pathologies. Prompt initiation and continuous administration of tocilizumab are likely key determinants of renal outcomes in such cases. In particular, when tocilizumab is suspended due to infection or in the perioperative period, consideration of its expeditious resumption should be made, taking into account both the withdrawal period and systemic conditions.
AB - Idiopathic multicentric Castleman disease (iMCD) is a systemic and polyclonal lymphoproliferative disease involving multiple organs, including the kidneys, due to the overproduction of interleukin-6 (IL-6). Recently, several reports have suggested that excessive IL-6 actions in iMCD could have a causal relationship with the development of diverse histopathological renal manifestations that cause nephrotic syndrome. However, the treatment for such cases remains unclear. We report a series of three cases of nephrotic syndrome due to iMCD that helps to delineate the importance of early and continuous therapy with the anti-interleukin-6 receptor antibody tocilizumab. First, treatment was suspended for infectious control, and the patient presented with nephrotic syndrome due to diffuse mesangial and endocapillary hypercellularity without immune deposits complicating acute kidney injury. Second, iMCD was treated with prednisolone alone. The patient suddenly developed nephrotic syndrome due to immune-complex glomerulonephritis, not otherwise specified, complicated with acute kidney injury. In the third case, nephrotic syndrome secondary to membranous glomerulonephritis was diagnosed, with a skin rash and IgE antibodies to tocilizumab, and was therefore treated with prednisolone alone. In contrast to the first two cases, the third progressed to end-stage renal disease on hemodialysis. Taken together, this series suggests that clinicians should maintain clinical vigilance for iMCD as a possible underlying component of nephrotic syndrome, since iMCD presents with a variety of renal pathologies. Prompt initiation and continuous administration of tocilizumab are likely key determinants of renal outcomes in such cases. In particular, when tocilizumab is suspended due to infection or in the perioperative period, consideration of its expeditious resumption should be made, taking into account both the withdrawal period and systemic conditions.
KW - IL-6 inhibitor
KW - acute kidney injury
KW - idiopathic multicentric Castleman disease
KW - renal pathology
KW - renal replacement therapy
KW - secondary nephrotic syndrome
KW - tocilizumab
UR - http://www.scopus.com/inward/record.url?scp=85146844211&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85146844211&partnerID=8YFLogxK
U2 - 10.3389/fmed.2022.1037032
DO - 10.3389/fmed.2022.1037032
M3 - Article
AN - SCOPUS:85146844211
SN - 2296-858X
VL - 9
JO - Frontiers in Medicine
JF - Frontiers in Medicine
M1 - 1037032
ER -