1. Challenges in primary focal segmental glomerulosclerosis diagnosis : from the diagnostic algorithm to novel biomarkers
- Author
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Jacobs-Cachá, Conxita, Vergara, Ander, García-Carro, Clara, Agraz Pamplona, Irene, Toapanta, Néstor, Ariceta Iraola, Gema, Moreso, Francesc, Serón, Daniel, López-Hellín, Joan, Soler, María José, Universitat Autònoma de Barcelona, Institut Català de la Salut, [Jacobs-Cachá C, García-Carro C, Agraz I, Moreso F, Serón D, Soler MJ] Grup de Recerca en Nefrologia, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Servei de Nefrologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. [Vergara A, Toapanta-Gaibor N] Grup de Recerca en Nefrologia, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Servei de Nefrologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. [Ariceta G] Red de Investigaciones Renales (RedInRen), Madrid, Spain. Servei de Nefrologia Pediàtrica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. [López-Hellín J] Red de Investigaciones Renales (RedInRen), Madrid, Spain. Servei de Bioquímica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. Grup de Recerca en Bioquímica, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain, and Vall d'Hebron Barcelona Hospital Campus
- Subjects
diagnosis algorithm ,030232 urology & nephrology ,Focal segmental glomerulosclerosis ,Disease ,030204 cardiovascular system & hematology ,Otros calificadores::Otros calificadores::/efectos adversos [Otros calificadores] ,urologic and male genital diseases ,Surgical Procedures, Operative::Surgical Procedures, Operative::Urogenital Surgical Procedures::Urologic Surgical Procedures::Kidney Transplantation [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT] ,03 medical and health sciences ,0302 clinical medicine ,Idiopathic nephrotic syndrome ,Glomerulopathy ,Ronyons - Malalties - Diagnòstic ,Other subheadings::Other subheadings::/adverse effects [Other subheadings] ,medicine ,Minimal change disease ,AcademicSubjects/MED00340 ,Otros calificadores::/terapia [Otros calificadores] ,Kidney transplantation ,CKJ Reviews ,focal segmental glomerulosclerosis ,Transplantation ,Kidney ,business.industry ,Primary Focal Segmental Glomerulosclerosis ,urogenital system ,Male Urogenital Diseases::Urologic Diseases::Kidney Diseases [DISEASES] ,Primary FSGS ,biomarkers ,Other subheadings::/therapy [Other subheadings] ,medicine.disease ,female genital diseases and pregnancy complications ,medicine.anatomical_structure ,intervenciones quirúrgicas::trasplante::trasplante de órganos::intervenciones quirúrgicas::trasplante de riñón [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS] ,Nephrology ,idiopathic nephrotic syndrome ,primary FSGS ,business ,enfermedades urogenitales masculinas::enfermedades urológicas::enfermedades renales [ENFERMEDADES] ,Nephrotic syndrome ,Algorithm ,Diagnosis algorithm ,Ronyons - Trasplantació - Complicacions ,Ronyons - Malalties - Tractament ,Biomarkers - Abstract
Biomarcadors; Algoritme de diagnosi; Glomerulosclerosi segmentària focal Biomarcadores; Algoritmo de diagnóstico; Glomeruloesclerosis segmentaria focal Biomarkers; Diagnosis algorithm; Focal segmental glomerulosclerosis Primary or idiopathic focal segmental glomerulosclerosis (FSGS) is a kidney entity that involves the podocytes, leading to heavy proteinuria and in many cases progresses to end-stage renal disease. Idiopathic FSGS has a bad prognosis, as it involves young individuals who, in a considerably high proportion (∼15%), are resistant to corticosteroids and other immunosuppressive treatments as well. Moreover, the disease recurs in 30–50% of patients after kidney transplantation, leading to graft function impairment. It is suspected that this relapsing disease is caused by a circulating factor(s) that would permeabilize the glomerular filtration barrier. However, the exact pathologic mechanism is an unsettled issue. Besides its poor outcome, a major concern of primary FSGS is the complexity to confirm the diagnosis, as it can be confused with other variants or secondary forms of FSGS and also with other glomerular diseases, such as minimal change disease. New efforts to optimize the diagnostic approach are arising to improve knowledge in well-defined primary FSGS cohorts of patients. Follow-up of properly classified primary FSGS patients will allow risk stratification for predicting the response to different treatments. In this review we will focus on the diagnostic algorithm used in idiopathic FSGS both in native kidneys and in disease recurrence after kidney transplantation. We will emphasize those potential confusing factors as well as their detection and prevention. In addition, we will also provide an overview of ongoing studies that recruit large cohorts of glomerulopathy patients (Nephrotic Syndrome Study Network and Cure Glomerulonephropathy, among others) and the experimental studies performed to find novel reliable biomarkers to detect primary FSGS. The authors are current recipients of research grants from the Fondo de Investigación Sanitaria-Feder, Instituto de Salud Carlos III (PI17/00257, PI18/01704 and PI18/01832) and REDinREN (RD16/0009/0030).
- Published
- 2020