Primary FSGS in Nephrotic Adults: Clinical Profile, Response to Immunosuppression and Outcome

Abstract

Aim: 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. Conclusion: 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.

Patterns of Primary Focal Segmental Glomerulosclerosis (FSGS) in Adults Treated in a Dakar Single Center: About 58 Cases

“…In our series, the urinary protein average was approximately in agreement with the various studies made on this subject [4] [8] [10] [12]. The classical form was the most frequently found histological type and this is in agreement with most of the studies [4] [8] [14].…”

“…We found a young population with an average age of 30.74 years. Similar results were found in Morocco, Tunisia and India with respective ages of 35.8, 39.9 and 31.1 years [4] [6] [7]. We noted a clear male predominance, which corroborated with most of the studies done in Africa, Asia and America [4] [7] [8] [9].…”

“…In Africa, it is the 2nd cause of primitive glomerulonephritis [6]. Early diagnosis and knowledge of the factors of poor prognosis is essential for monitoring patients [4].…”

“…Steroid-dependence, steroid-resistance and relapses are frequent and require a combination with immunosuppressive therapy [3]. The potential risk of CNI-induced nephrotoxicity and the cost of that therapy are a major deterrent for patients in developing countries [4]. The aim of our study was to assess our practice in relation to this lesion by determining the clinical, pathological, therapeutic and evolutive aspects.…”

Diagnóstico de infección por VIH por el hallazgo incidental de alteraciones ultraestructurales en una biopsia renal: Report of one case

“…También, la ultraestructura mostró fusión pedicelar muy extensa, lo que explica la magnitud de la proteinuria. Esta alteración no es propia de la EMBD sino que se observa característicamente en formas primarias de enfermedades podocitarias 15 . En este paciente es planteable, dada su gran masa corporal, que esta alteración podocitaria sea una manifestación incipiente de una glomeruloesclerosis focal y segmentaria (GEFS) secundaria a su desproporción entre masa corporal/masa renal.…”