Crystal-Induced Podocytopathy Producing Collapsing Focal Segmental Glomerulosclerosis in Monoclonal Gammopathy of Renal Significance: A Case Report.
Focal and segmental glomerulosclerosis
crystalline keratopathy
crystalloid inclusions
plasma cell dyscrasia
podocytopathy
Journal
Kidney medicine
ISSN: 2590-0595
Titre abrégé: Kidney Med
Pays: United States
ID NLM: 101756300
Informations de publication
Date de publication:
Historique:
entrez:
17
8
2021
pubmed:
18
8
2021
medline:
18
8
2021
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 34401732
doi: 10.1016/j.xkme.2021.03.007
pii: S2590-0595(21)00096-0
pmc: PMC8350840
doi:
Types de publication
Case Reports
Langues
eng
Pagination
659-664Commentaires et corrections
Type : ErratumIn
Informations de copyright
© 2021 The Authors.
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