Optimal management of primary focal segmental glomerulosclerosis in adults
نویسندگان
چکیده
Focal segmental glomerulosclerosis (FSGS) is a frequent glomerular kidney disease that is revealed by proteinuria or even nephrotic syndrome. A diagnosis can be established from a kidney biopsy that shows focal and segmental glomerulosclerosis. This histopathological lesion may be caused by a primary podocyte injury (idiopathic FSGS) but is also associated with other pathologies (secondary FSGS). The first-line treatment for idiopathic FSGS with nephrotic syndrome is a prolonged course of corticosteroids. However, steroid resistance or steroid dependence is frequent, and despite intensified immunosuppressive treatment, FSGS can lead to end-stage renal failure. In addition, in some cases, FSGS can recur on a graft after kidney transplantation: an unidentified circulating factor may be implicated. Understanding of its physiopathology is unclear, and it remains an important challenge for the scientific community to identify a specific diagnostic biomarker and to develop specific therapeutics. This study reviews the treatment of primary FSGS and the recurrence of FSGS after kidney transplantation in adults.
منابع مشابه
Is focal segmental glomerulosclerosis really focal? Distribution of lesions in adults and children.
The distribution of lesions of glomerulosclerosis, whether focal or diffuse, has important implications for pathogenesis and potential therapeutic response. Determination of focal or diffuse nature of lesions from a single section, may, however, be misleading. We therefore evaluated the distribution of segmental glomerulosclerosis in patients with nephrotic syndrome and idiopathic focal segment...
متن کاملThe predictive role of histopathological findings in renal insufficiency and complete remission in a sample of Iranian adults with primary focal segmental glomerulosclerosis
BACKGROUND Primary focal segmental glomerulosclerosis (FSGS) is defined by the presence of proteinuria, often in nephrotic range and pathologically by segmental scars (SS). The aim of this study is to identify the possible predictors of complete remission or progression to chronic kidney disease in Iranian adults with primary focal segmental glomerulosclerosis. METHODS In this historical coho...
متن کاملPOEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link
BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein skin changes (POEMS) syndrome is a rare cause of polyneuropathy. Calciphylaxis, a severe disease leading to necrotic ulcers of the skin, is associated with POEMS syndrome and also with renal disease. This case report describes a patient with POEMS syndrome plus primary focal segmental glomerulosclerosis. CASE PRESENTATION A 27-ye...
متن کاملClinicopathological correlation and treatment response of primary focal segmental glomerulosclerosis in adults and adolescents
The incidence of focal segmental glomerulosclerosis (FSGS) is approximately 10% in children <6 years, 20% in adolescents, and 20-25% in adults. A retrospective observational study was done to document clinicopathological correlation, treatment response, and risk factors in the progression of chronic kidney disease (CKD) of primary FSGS in adults and adolescents. A total of 170 patients were stu...
متن کاملKidney transplantation for primary focal segmental glomerulosclerosis: outcomes and response to therapy for recurrence.
BACKGROUND For a subset of adults and children with primary focal segmental glomerulosclerosis (FSGS), proteinuria and renal dysfunction recur after kidney transplantation (KTx). Predicting recurrence and response to plasmapheresis (PP) or other interventions remains problematic. METHODS The prevalence, recurrence rate, outcomes, and treatment responses of patients with FSGS were determined a...
متن کامل