Focal segmental glomerulosclerosis associated with cutaneous and systemic plasmacytosis

Abstract

Cutaneous and systemic plasmacytosis (CSP) is a rare lymphoproliferative disorder that mainly affects middle-aged Asian individuals. Although Castleman disease is often complicated with various renal involvements, glomerulonephritis associated with CSP, which is considered as a variant of Castleman disease, is rare. This report presents the case of a 41-year-old Japanese man with nephrotic syndrome associated with CSP. Renal biopsy findings showed focal segmental glomerulosclerosis (FSGS) and diffusely mild segmental mesangial proliferation. Plasma cell infiltration in the interstitium was not observed. Electron microscopic findings showed diffuse foot process effacement, localized involvement of subendothelial space widening with amorphous materials, and endothelial cell swelling. Lymph node biopsy findings denied Castleman disease. His skin regions and proteinuria were successfully treated with prednisolone and cyclosporine. The causal relationship between CSP and FSGS is unknown. However, increased serum levels of IL-6 and VEGF and decreased VEGF expression in the podocyte may contribute to renal lesions in patients with CSP. To our best knowledge, this is the first case of a patient with FSGS associated with CSP.

STAT-3 signaling role in an experimental model of nephropathy induced by doxorubicin

“…Thus, it is possible to infer that DOX causes in ammation, with a possible in ltration of macrophages, which in our study can be represented by elevated index of in ammatory cells in renal tissues. These cells are responsible to increase of IL-6 mRNA expression that is frequently observed in patients with primary FSGS associated with cutaneous and systemic plasmacytosis [42], beyond other nephropathies, such as IgA nephropathy [43]. In line with this, it was also detected signi cant differences in IL-6 receptor gene (IL-6R) expression between the DOX-injected and STATTIC-treated animals.…”

Novel Diagnostic and Therapeutic Techniques Reveal Changed Metabolic Profiles in Recurrent Focal Segmental Glomerulosclerosis 

“…Immunohistochemically staining of human kidney biopsy specimens indicated that the expression of cathepsin D was signi cantly increased in Minimal change disease compared to that in FSGS maybe because of a high level of autophagic activity 38,39 . IL6 was demonstrated to contribute to renal diseases like FSGS 40 . Cathepsin L and IL6 were undetectable or in normal range in in our patient.…”

Novel diagnostic and therapeutic techniques reveal changed metabolic profiles in recurrent focal segmental glomerulosclerosis

“…Therefore, we do not believe that CLCF1 is specific for recurrent FSGS and most likely was not the Immunohistochemically staining of human kidney biopsy specimens indicated that the expression of cathepsin D was significantly increased in Minimal change disease compared to that in FSGS maybe because of a high level of autophagic activity 40,41 . IL6 was demonstrated to contribute to renal diseases like FSGS 42 . Cathepsin L and IL6 were undetectable or in normal range in our patient.…”