title: Primary FSGS in Nephrotic Adults: Clinical Profile, Response to Immunosuppression and Outcome.
abstract: OBJECTIVE
  Primary focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic proteinuria in adults. Most studies on FSGS have combined pediatric and adult patients. This study aims at assessing the response to immunosuppression and its impact on renal survival in adults with primary FSGS.
  
  
  METHODS
  Patients with nephrotic proteinuria with primary FSGS seen from January 2010 to December 2014 were included. Clinical, laboratory and treatment details were recorded. Deterioration in renal function was defined as ≥50% decline in estimated glomerular filtration rate (eGFR) or progression to end-stage renal disease.
  
  
  RESULTS
  There were 116 patients with median follow-up of 23.6 (6-65.1) months. Baseline proteinuria was 5.1 ± 2.6 g/day and eGFR was 96.9 ± 35.1 ml/min/1.73 m2. One hundred one (94.4%) patients had received angiotensin converting enzyme inhibitor/angiotensin receptor blocker (ACEi/ARB). One hundred fourteen patients received steroids. Forty two of 114 patients (36.8%)were steroid resistant. Thirty eight received calcineurin inhibitors (CNI). Seventeen (44.7%) were CNI resistant of which 2 achieved remission with alternate immunosuppression. Eleven (9.5%) patients had worsening renal function - 9 had no remission, 2 had PR with none in CR (30 vs. 5.6% vs. 0, respectively, log-rank, p < 0.001). ACEi/ARBs use and remission of proteinuria were independently associated with better renal survival.
  
  
  CONCLUSIONS
  Achieving remission, whether complete or partial, is the critical factor in predicting renal survival in nephrotic adults with primary FSGS. Steroid-resistant patients have reasonable renal survival, if proteinuria is reduced with timely use of alternate immunosuppression. CNI resistance is a major hurdle in management with limited treatment options.
authors.affiliation: Department of Nephrology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Soumita
authors.initials: S
authors.lastname: Bagchi
authors.forename: Sanjay
authors.initials: S
authors.lastname: Agarwal
authors.forename: Mani
authors.initials: M
authors.lastname: Kalaivani
authors.forename: Dipankar
authors.initials: D
authors.lastname: Bhowmik
authors.forename: Geetika
authors.initials: G
authors.lastname: Singh
authors.forename: Sandeep
authors.initials: S
authors.lastname: Mahajan
authors.forename: Amit
authors.initials: A
authors.lastname: Dinda
mesh_terms: D000293:Adolescent; D000328:Adult; D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate; D005923:Glomerulosclerosis, Focal Segmental / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007668:Kidney / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000503:physiopathology; D007677:Kidney Function Tests; D008297:Male; D008875:Middle Aged; D009401:Nephrosis / Q000150:complications / Q000628:therapy*; D011507:Proteinuria / Q000209:etiology / Q000628:therapy; D014022:Tissue Survival; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers; D000806:Angiotensin-Converting Enzyme Inhibitors; D007166:Immunosuppressive Agents