title: The treatment of idiopathic focal segmental glomerulosclerosis in adults.
abstract: Focal segmental glomerulosclerosis (FSGS) is the histologic end point of many disease processes that affect the kidney. Clinically, adults with FSGS present with proteinuria that may be accompanied by the nephrotic syndrome. Once identifiable (secondary) causes are excluded, the diagnosis of idiopathic FSGS, a challenging glomerular disease to understand and manage, is made. On the basis of mostly retrospective data, first-line treatment for idiopathic FSGS patients with nephrotic-range proteinuria is a prolonged course of corticosteroids. However, steroid resistance is common and portends an increased risk of long-term decline in kidney function and end-stage kidney disease in these patients compared with responders. Multiple other immunosuppression regimens have been used in steroid-resistant FSGS, some of which have been studied in randomized controlled trials. Here, we review the data on the treatment for idiopathic FSGS in adults.
authors.affiliation: Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, NY. Electronic address: jjh2165@columbia.edu.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Hogan
authors.affiliation: Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, NY.
authors.forename: Jai
authors.initials: J
authors.lastname: Radhakrishnan
mesh_terms: D000328:Adult; D057912:Angiotensin II Type 2 Receptor Blockers / Q000627:therapeutic use*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000188:drug therapy*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006973:Hypertension / Q000150:complications / Q000188:drug therapy*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D009404:Nephrotic Syndrome / Q000150:complications / Q000188:drug therapy*; D011507:Proteinuria / Q000150:complications / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D057912:Angiotensin II Type 2 Receptor Blockers; D000806:Angiotensin-Converting Enzyme Inhibitors; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid
keywords: Focal segmental glomerulosclerosis