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Crystal-induced podocytopathy producing collapsing focal segmental glomerulosclerosis in monoclonal gammopathy of renal significance: a case report

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dc.contributor.author Buxeda, Anna
dc.contributor.author Said, Samar
dc.contributor.author Nasr, Samih H.
dc.contributor.author Soler, María José
dc.contributor.author Howard, Mathew T.
dc.contributor.author Maguire, Leo J.
dc.contributor.author Fervenza, Fernando C.
dc.date.accessioned 2022-06-27T06:36:50Z
dc.date.available 2022-06-27T06:36:50Z
dc.date.issued 2021
dc.identifier.citation Buxeda A, Said S, Nasr SH, Soler MJ, Howard MT, Maguire LJ, et al. Crystal-induced podocytopathy producing collapsing focal segmental glomerulosclerosis in monoclonal gammopathy of renal significance: a case report. Kidney Med. 2021 May 14; 3(4): 659-64. DOI: 10.1016/j.xkme.2021.03.007
dc.identifier.issn 2590-0595
dc.identifier.uri http://hdl.handle.net/10230/53602
dc.description.abstract Monoclonal gammopathy-associated crystalline podocytopathy causing collapsing focal segmental glomerulosclerosis (FSGS) is very rare and has been associated with pamidronate therapy. We present the case of a 53-year-old man with vision loss secondary to corneal crystals deposition, nephrotic-range proteinuria, and reduced glomerular filtration rate without associated comorbid conditions. Two kidney biopsies were initially reported as primary FSGS but the patient did not respond to high-dose corticosteroid immunosuppression therapy. Repeat review of biopsies with additional electron microscopy analysis revealed crystalline inclusions in podocytes leading to collapsing FSGS. Subsequent workup revealed an immunoglobulin G κ serum monoclonal protein. Bone marrow biopsy revealed 5% κ-restricted plasma cells with cytoplasmic crystalline inclusions. To our knowledge, this is the first case of monoclonal gammopathy of clinical significance manifesting as crystalline podocytopathy leading to collapsing FSGS and keratopathy leading to vision loss. Crystalline podocytopathy should be considered in the differential diagnosis of collapsing glomerulopathy, and careful ultrastructural examination of the kidney biopsy specimen is crucial to establish this diagnosis.
dc.format.mimetype application/pdf
dc.language.iso eng
dc.publisher Elsevier
dc.rights Copyright © 2021 The Authors. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
dc.rights.uri http://creativecommons.org/licenses/by-nc-nd/4.0/
dc.title Crystal-induced podocytopathy producing collapsing focal segmental glomerulosclerosis in monoclonal gammopathy of renal significance: a case report
dc.type info:eu-repo/semantics/article
dc.identifier.doi http://dx.doi.org/10.1016/j.xkme.2021.03.007
dc.subject.keyword Focal and segmental glomerulosclerosis
dc.subject.keyword Crystalline keratopathy
dc.subject.keyword Crystalloid inclusions
dc.subject.keyword Plasma cell dyscrasia
dc.subject.keyword Podocytopathy
dc.rights.accessRights info:eu-repo/semantics/openAccess
dc.type.version info:eu-repo/semantics/publishedVersion

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