Background and Aims We aimed to investigate the characteristics and survival data of biopsy-proven primary focal segmental glomerulosclerosis (FSGS) in adult patients across Turkey. Method Patients with primary FSGS were included by retrospectively scanning the database of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD). Demographic and laboratory data of the patients at baseline, sixth month, first year, and third year were recorded. Patients with secondary FSGS, missing data were excluded. Results The study included 1668 patients with primary FSGS who met the criteria. 1386 patients were included. The mean age of the patients was 41.16±13.88 years, and 712 patients (51.4%) were male. The total follow-up period from the biopsy date was 37.63±40.45 (IQR:1-249) months. The mean blood pressure of the patients, respectively; 130.43±17.63/81.47±10.85 mmHg, serum creatinine 1.29±1.28 mg/dl, e-GFR: 86.10±42.70 ml/min/1.73 m2, serum albumin: 3.41±0.92 g/dl and proteinuria amount was 4687±4658 g/day. Microscopic hematuria was detected in 40.2% of the patients. The rate of admission with nephrotic syndrome was .45.7%. In light microscopy, the mean glomeruli count was 17.36±10.58, with 3.32±4.08 global sclerosis and 0.08±065 glomeruli had segmental sclerosis. Mesangial proliferation was found in 53.1% of the patients and interstitial inflammation was found in 69.7% of the patients. Interestingly, the most common immunoglobulin staining was IgM (19.3%) in the immunofluorescent microscope. The rate of receiving immunosuppressive therapy was 36%. A positive correlation was found in terms of serum creatinine, albumin, and proteinuria in the 3-year follow-up (p<0.001). In the univariate analysis, the group with e-GFR<60 ml/min/1.73 m2 was older, hypertensive, uremic, anemic, had more interstitial fibrosis/tubular atrophy and less interstitial inflammation and mesangial proliferation (p<0.001). In terms of quantitative proteinuria, Patients with proteinuria >3.5 g/day were more hypertensive, hyperlipidemic, hypoalbuminemic and anemic (p<0.05). Conclusion Our study presented important data on the status of patients with national primary FSGS. Approximately one-third of patients receive immunosuppressive therapy. The most important factors determining the prognosis of primary FSGS are the initial nephrotic proteinuria and the degree of renal function.
Background and Aims IgA nephropathy (IgAN) is the most common cause of primary glomerulonephritis in Turkey, as well as all over the world. Along with the frequent occurrence, deleterious renal outcome odds make treatment approaches important. Additionally, for high-risk individuals immunosuppressive treatment (IST) is recommended. However, studies to date revealed conflicting results regarding IST. Therefore, we aimed to investigate IST results among IgAN patients which is the leading primary glomerulonephritis in Turkiye. Method The data of 1656 IgAN patients in the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD) were analyzed. A total of 506 primary IgAN patients (63.4% male, mean age 38.9±12.5 years) were included and divided into two groups according to treatment protocols as isolated corticosteroid (69.6%) and combined IST (30.4%) groups. The median follow-up duration was 24 (3-218) months. Results Remission (66.6% partial remission, 33.4% complete remission) was achieved in 70.6% of patients. Systolic and diastolic blood pressures, urea, creatinine, and proteinuria levels were lower, and eGFR levels were higher in responsive patients (Table 1). There was no difference between the treatment groups in terms of remission rates (p = 0.147) and remission types’ rates (p = 0.279). Remission rates were different between treatment subgroups. However, there was no difference between the treatment subgroups according to the remission types (p = 0.132) (Table 2). Complete remission was lower in the S1 and T1 categories (p = 0.003 and 0.039, respectively). The serious infection was higher in the combined IST group (17.1% vs 2.9%). The outcome data of 229 individuals was evaluated, 40 of 229 (17.5%) developed ESRD and 8 were dead. In the multivariate analysis, eGFR (OR 1.007, 95%CI 1.001-1.013, p = 0.020), proteinuria (OR 1.000, p = 0.009), MEST-C S1 (OR 1.912, 95%CI 1.216-3.005, p = 0.005), MEST-C T2 (OR 0.226, 95%CI 0.102-0.501, p = < 0.001) were found to be significant regarding remission. Conclusion IST provides remission in high-risk IgAN patients but was associated with serious adverse events. The fact that the remission rates were similar between the treatment groups and that the complete remission rate was low in chronic changes supports the necessity of determining the treatment choice according to patient characteristics.
Objective: Amyloidosis is disorder of various etiologies in which abnormally folded fibrillary protein deposits with more than thirty forms infiltrate into extracellular spaces of affected organs. Renal involvement is clinically characterized by decreased estimated glomerular filtration rate (eGFR) and proteinuria. The aim of present study was to classify and grade renal amyloidosis cases using renal amyloid prognostic score (RAPS) systems, correlate clinical data and chronic kidney disease (CKD) stages. Methods: We retrospectively analyzed kidney biopsies of 45 patients diagnosed with renal amyloidosis applied between 2017-2022 to our department and scored each of patients according to RAPS. Results: 8.9% of patients had RAPS score 1, 53.3% had 2 and 37.8% had 3. Urea, serum creatinine and proteinuria levels of RAPS3 patients were significantly higher and eGFR levels were lower compared to RAPS1 patients (p<0.01). According to CKD stages, no significant difference was observed in glomerular amyloid deposition class and score, vascular and interstitial amyloid deposition scores, and glomerular sclerosis (p>0.05). The interstitial fibrosis, inflammation values and RAPS scores were found to be significantly higher in advanced CKD stages (p<0.05). Majority of patients at CKD stage 1-2 had RAPS score 2 (73.68%), while 57.1% of at stage 3 and 66.7% at stage 4-5 had RAPS score of 3 (p=0.0015). Conclusion: As a result, the intestinal fibrosis, inflammation values, RAPS scores were significantly higher in advanced CKD stages. Distribution pattern of amyloid in the renal parenchyma compartment, grade of RAPS and eGFR were associated with urea/creatinine, proteinuria levels and thus with CKD stage.
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