Sistema de Salut de Catalunya
Institut Català de la Salut
[Buxeda A] Division of Nephrology and Hypertension, Mayo Clinic College of Medicine, Rochester, MN. Division of Nephrology, Hospital del Mar, Barcelona, Spain. [Said S, Samih H. Nasr] Division of Anatomic Pathology, Mayo Clinic College of Medicine, Rochester, MN. [Soler MJ] Servei de Nefrologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Howard MT] Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, MN. [Maguire LJ] Department of Ophthalmology, Mayo Clinic College of Medicine, Rochester, MN
Vall d'Hebron Barcelona Hospital Campus
2022-02-22T07:41:55Z
2022-02-22T07:41:55Z
2021-07
Glomeruloesclerosis focal y segmentaria; Queratopatía cristalina; Podocitopatía
Glomeruloesclerosi focal i segmentària; Queratopatia cristal·lina; Podocitopatia
Focal and segmental glomerulosclerosis; Crystalline keratopathy; Podocytopathy
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. Re-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.
The authors declare that they have no relevant financial interests.
Article
Published version
English
Glomèruls renals - Malalties - Complicacions; Gammapaties monoclonals - Complicacions; DISEASES::Male Urogenital Diseases::Urologic Diseases::Kidney Diseases::Nephritis::Glomerulonephritis::Glomerulosclerosis, Focal Segmental; Other subheadings::Other subheadings::Other subheadings::/complications; DISEASES::Hemic and Lymphatic Diseases::Hematologic Diseases::Blood Protein Disorders::Hypergammaglobulinemia::Monoclonal Gammopathy of Undetermined Significance; Other subheadings::Other subheadings::Other subheadings::/complications; ENFERMEDADES::enfermedades urogenitales masculinas::enfermedades urológicas::enfermedades renales::nefritis::glomerulonefritis::glomeruloesclerosis focal; Otros calificadores::Otros calificadores::Otros calificadores::/complicaciones; ENFERMEDADES::enfermedades hematológicas y linfáticas::enfermedades hematológicas::trastornos de las proteínas sanguíneas::hipergammaglobulinemia::gammopatía monoclonal de relevancia indeterminada; Otros calificadores::Otros calificadores::Otros calificadores::/complicaciones
Elsevier
Kidney Medicine;3(4)
https://doi.org/10.1016/j.xkme.2021.03.007
Attribution-NonCommercial-NoDerivatives 4.0 International
http://creativecommons.org/licenses/by-nc-nd/4.0/
Articles científics - HVH [3439]
