Clinical and histopathological characteristics of primary focal segmental glomerulosclerosis in Turkish adults.

The data regarding primary FSGS (pFSGS) from different parts of the world differ. While the prevalence of pFSGS has been increasing in Western countries like the USA, it follows an inconsistent trend in Europe and Asia and a decreasing trend in Far Eastern countries such as China in the last two decades. There are undetermined factors to explain those national and geographic discrepancies. Herein, we aimed to reveal the current prevalence with clinical and histopathological characteristics of pFSGS in Turkish adults. This study includes the biopsy-proven pFSGS patients data recorded between 2009 and 2019, obtained from the national multicenter primary glomerulonephritis registry system of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database. 850 of the 3875 primer glomerulonephritis patients(21.9%) have pFSGS. The mean age is 40.5 ± 14.2 and 435 (51.2%) of patients are male. Nephrotic syndrome is the most common biopsy indication (59.2%). 32.6% of patients have hematuria, 15.2% have leukocyturia and 7.8% have both. Serum creatinine, albumin, and proteinuria are 1.0 mg/dL (IQR = 0.7-1.4) mg/dl, 3.4 ± 0.9 g/dl, 3400 mg/day(IQR, 1774-5740), respectively. Females have lower mean arterial pressure (- 2.2 mmHg), higher eGFR (+ 10.0 mL/min/1.73 m2), and BMI (+ 1.6 kg/m2) than males. Thickened basal membrane(76.6%) and mesangial proliferation (53.5%) on light microscopy are the major findings after segmental sclerosis. IgM (32.7%) and C3 (32.9%) depositions are the most common findings on immunofluorescence microscopy. IgM positivity is related to lower eGFR, serum albumin, and higher proteinuria. The prevalence of pFSGS is stable although slightly increasing in Turkish adults. The characteristics of the patients are similar to those seen in Western countries.

Could mesangial C3 deposition be an independent prognostic marker in immunoglobulin A nephropathy?

BACKGROUND: Immunoglobulin A nephropathy (IgAN) is a common primary glomerulonephropathy. There is evidence that mesangial C3 deposition plays a role in the development of the disease. The aim of this study was to examine the effect of C3 deposition on the prognosis of IgAN patients. METHOD: The study included 1135 patients with biopsy-confirmed IgAN from the database of the Turkish Nephrology Association Glomerular Diseases Working Group (TSN-GOLD). Patients were excluded from the study if they were aged < 18 or > 75 years or if C3 staining had not been performed in the immunofluorescent analysis. C3 deposition was defined as an immunofluorescence intensity of C3 ≥ 2 + within the mesangium. The primary endpoints were the development of end-stage renal disease, a 30% decrease in glomerular filtration rate compared to the basal value or an elevation in proteinuria to a nephrotic level (3.5 gr/day). RESULTS: Mesangial C3 deposition was observed in 603 (53.1%) patients. No statistically significant difference was found at baseline between the groups with and without mesangial C3 deposition, as for age, sex, BMI, proteinuria level, or the presence of hypertension. In the follow-up period with a mean duration of 78 months, no significant difference was found between the two groups regarding the primary endpoints (p = 0.43). A significant correlation between C3 deposition and segmental glomerulosclerosis (S1) according to the Oxford MEST-C classification was found (p = 0.001). CONCLUSION: Although a correlation was observed between mesangial C3 deposition and the S1 MEST-C classification, mesangial C3 deposition was not a prognostic factor in IgAN.

Demographic, clinical and laboratory characteristics of adult-onset minimal change disease in Turkey: Turkish Society of Nephrology-Glomerular Diseases (TSN-GOLD) Working Group.

PURPOSE: In our study, diagnostic and demographic characteristics of patients diagnosed with minimal change disease (MCD) by biopsy, clinical and laboratory findings in our country were investigated. METHODS: Data were obtained from the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group database. Demographic characteristics, indications for biopsy, diagnosis of the glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were recorded. The data presented are cross-sectional and includes application data for the biopsy period. RESULTS: Of 3875 patients, 233 patients with MCD (median age 35.0 years) were included in the study, which constitutes 6.0% of the total glomerulonephritis database. Renal biopsy was performed in 196 (84.1%) patients due to nephrotic syndrome. Median serum creatinine was 0.7 (0.6-1.0) mg/dl, mean eGFR was 104 ± 33 ml/min/1.73 m2 and median proteinuria 6000 mg/day. The number of patients under the age of 40 years was 139 (59.7%) (Group A), and the number of patients aged 40 years and over was 94 (40.3%) (Group B). Compared to Group A, global sclerotic glomeruli (24 vs. 43, p < 0.001) interstitial inflammation (15 vs. 34, p < 0.001), interstitial fibrosis (20 vs. 31, p = 0.001, vascular changes (10 vs. 25, p < 0.001) and tubular atrophy (18 vs. 30, p < 0.001) were found to be significantly higher in Group B. There was no difference in immunofluorescent staining properties between the two groups. CONCLUSION: Our data are generally compatible with the literature. Chronic histopathological changes were more common in patients aged 40 years and older than younger patients. Studies investigating the effects of these different features on renal survival are needed.

Prognostic value of histopathological scoring and grading in patients with renal AA amyloidosis.

OBJECTIVE: The amount and distribution pattern of amyloid deposits may contribute to renal function and outcome, given the great diversity of renal involvement in amyloidosis. The aim of this study was to analyze the impact of histological characteristics of patients with biopsy-proven renal AA amyloidosis (AAA) on renal outcome. METHODS: Renal biopsies of 37 patients with AAA were re-evaluated. The distribution pattern of glomerular amyloid (GA) deposits was classified, the extent of amyloid deposits in glomeruli, vessel, and interstitium and other histopathologic lesions were scored, and renal amyloid prognostic score (RAPS) was determined by summing all scores. Their potential prognostic relevance on renal outcome was investigated. RESULTS: GA and vascular amyloid (VA) deposits were noted in all cases, interstitial amyloid (IA) was detected in 70.2%. GA deposits were predominantly seen in diffuse mesengiocapillary fashions (class IV) (51.4%). GA class, the extent of GA, VA, IA deposit, and RAPS, as well as interstitial fibrosis (IF) and interstitial inflammation were correlated to renal function at diagnosis. During the median follow-up of 52 months, 13 patients developed doubling of serum creatinine or end stage renal disease and they had a higher degree of GA and VA load (p = 0.03 and p = 0.042, respectively) as compared to the remaining patients. VA load, but not GA and RAPS grade, was associated with poor renal outcome (HR 3.016, 95% CI 1.45-6.25, p = 0.003). CONCLUSIONS: Baseline renal function is closely linked to the extent of AA amyloid deposit in renal parenchyma but only VA load was a predictor of renal outcome in AAA patients.

Spectrum of biopsy-based renal disease in an elderly Turkish population.

AIM: The prevalence of kidney disease and the number of renal biopsies performed in the elderly are increasing with aging of the population. We aimed to analyze the clinicopathological findings of kidney diseases in the elderly (≥ 65 years), in comparison with their younger counterparts (aged 16 - 64 years). MATERIALS AND METHODS: The data at the time of renal biopsy were obtained by reviewing medical records and the biopsy reports of the patients retrospectively. The data at the time of renal biopsy were obtained by reviewing medical records and the biopsy reports of the patients who underwent renal biopsy at Bakirkoy Dr. Sadi Konuk Education and Research Hospital. Demographic characteristics, clinical syndrome at presentation, and histopathological diagnosis of all patients were recorded. RESULTS: Among the 750 renal biopsies, 93 were performed in elderly patients, which constitutes 12.4% of the biopsies. The mean age of the patients was 71.7 ± 5.4 years, and 62.4% were male. The most common indication for renal biopsy was nephrotic syndrome (NS) in elderly and younger age groups (45.2% vs. 40%). The most frequent histopathological diagnosis was membranous glomerulonephritis (MGN). According to the clinical presentation, MGN (42.8%) was the leading cause of NS, and almost 1/3 of the patients with acute or rapidly progressive renal failure were found to have pauci-immune glomerulonephritis (GN). In comparison with renal biopsy results of younger patients, MGN and pauci-immune GN were more prevalent, but IgA nephropathy, lupus nephritis, and acute tubular necrosis were less common in elderly patients. CONCLUSION: Clinical presentation and the frequency of certain renal pathologies differ with age. The relatively high prevalence of potentially curative kidney diseases in the elderly indicates the importance of renal biopsy in these patients.

The relationship between glomerular IgG staining and poor prognostic findings in patients with IgA nephropathy: the data from TSN-GOLD working group.

BACKGROUND: Galactose-deficient IgA1 (Gd-IgA1) has an increased tendency to form immunocomplexes with IgG in the serum, contributing to IgAN pathogenesis by accumulating in the glomerular mesangium. Several studies showed that glomerular IgG deposition in IgAN is an important cause of mesangial proliferation and glomerular damage. This study aims to determine the association of the positivity of IgG and the intensity of IgG staining with a poor renal prognosis. METHODS: A total of 943 IgAN patients were included in the study. Glomerular IgG staining negative and positive patients were compared using Oxford classification scores, histopathological evaluations, proteinuria, eGFR, albumin, blood pressures. IgG positive patients were classified as (+), (++), (+++) based on their staining intensity, and the association with the prognostic criteria was also evaluated. RESULTS: 81% (n = 764) of the patients were detected as IgG negative, while 19% (n = 179) were positive. Age, gender, body mass index, blood pressure, proteinuria, eGFR, uric acid values were similar in IgG positive and negative patients who underwent biopsy (p > 0.05). Intensity of glomerular IgG positivity was not found to be associated with diastolic and systolic blood pressure, urea, uric acid, age, eGFR, albumin, proteinuria (p > 0.05 for all, r = - 0.084, r = - 0.102, r = - 0.006, r = 0.062, r = 0.014, r = - 0.044, r = - 0.061, r = - 0.066, r = 0.150, respectively). There was no difference for histopathological findings between IgG (+), IgG (++), IgG (+++) groups (for all, p > 0.05). CONCLUSION: Glomerular IgG negativity and positivity detected by routine IFM in IgAN patients is not associated with poor renal prognostic risk factors.

Characteristics of primary glomerular diseases patients with hematuria in Turkey: the data from TSN-GOLD Working Group.

PURPOSE: Hematuria is one of the most common laboratory findings in nephrology practice. To date, there is no enough data regarding the clinical and histopathologic characteristics of primary glomerular disease (PGD) patients with hematuria in our country. METHODS: Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database between May 2009 and June 2019. The data of all PGD patients over the age of 16 years who were diagnosed with renal biopsy and had hematuria data were included in the study. Demographic characteristics, laboratory and biopsy findings were also recorded. RESULTS: Data of 3394 PGD patients were included in the study. While 1699 (50.1%) patients had hematuria, 1695 (49.9%) patients did not have hematuria. Patients with hematuria had statistically higher systolic blood pressure, serum blood urea nitrogen, creatinine, albumin, levels and urine pyuria. However, these patients had statistically lower age, body mass index, presence of hypertension and diabetes, eGFR, 24-h proteinuria, serum total, HDL and LDL cholesterol, and C3 levels when compared with patients without hematuria. Hematuria was present 609 of 1733 patients (35.8%) among the patients presenting with nephrotic syndrome, while it was presented in 1090 of 1661 (64.2%) patients in non-nephrotics (p < 0.001). CONCLUSION: This is the first multicenter national report regarding the demographic and histopathologic data of PGD patients with or without hematuria. Hematuria, a feature of nephritic syndrome, was found at a higher than expected in the PGDs presenting with nephrotic syndrome in our national database.

Epidemiological features of primary glomerular disease in Turkey: a multicenter study by the Turkish Society of Nephrology Glomerular Diseases Working Group.

BACKGROUND: The largest data on the epidemiology of primary glomerular diseases (PGDs) are obtained from the databases of countries or centers. Here, we present the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group. METHODS: Data of patients who underwent renal biopsy and received the diagnosis of PGD were recorded in the database prepared for the study. A total of 4399 patients from 47 centers were evaluated between May 2009 and May 2019. The data obtained at the time of kidney biopsy were analyzed. After the exclusion of patients without light microscopy and immunofluorescence microscopy findings, a total of 3875 patients were included in the study. RESULTS: The mean age was 41.5 ± 14.9 years. 1690 patients were female (43.6%) and 2185 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGD was IgA nephropathy (25.7%) followed by membranous nephropathy (25.6%) and focal segmental glomerulosclerosis (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. The mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. The median proteinuria, serum creatinine, estimated GFR, and mean albumin values were 3300 (IQR: 1467-6307) mg/day, 1.0 (IQR: 0.7-1.6) mg/dL, 82.9 (IQR: 47.0-113.0) mL/min and 3.2 ± 0.9 g/dL, respectively. CONCLUSIONS: The distribution of PGDs in Turkey has become similar to that in other European countries. IgA nephropathy diagnosed via renal biopsy has become more prevalent compared to membranous nephropathy.

Leptospirosis With Acute Renal Failure and Vasculitis: A Case Report.

Leptospirosis is a zoonotic disease that occurs worldwide. Various clinical manifestations of leptospirosis can be seen. In this article, we present a case with acute renal failure, severe vasculitis and hyperbilirubinemia occurring simultaneously with leptospirosis. A 45-year-old male patient presented with fever, myalgia, jaundice and reddish skin lesions and anuria. Physical examination findings were icteric sclera, large tender hepatomegaly, and lower extremities' cutaneous necrosis due to vasculitis. Hemodialysis was started. Kidney biopsy revealed degenerative changes of proximal tubules, some of them containing bile casts. Microscopic agglutination test was positive and consistent with leptospirosis. Intravenous ampicillin and oral tetracycline were started. Methylprednisolone 60 mg per day was given for skin vasculitis. Hemodialysis therapy was discontinued. All clinical findings gradually regressed.

Interferon ß associated nephropathy in a Multiple Sclerosis patient: A case and review.

BACKGROUND: Interferon beta (IFN ß) subtypes are largely used as immunomodulatory agents in Multiple Sclerosis (MS) treatment. While being generally well tolerated, they can cause various side effects. Adverse effects related to kidney are rarely reported. CASE REPORT: We report a 32 years old male patient who developed nephrotic syndrome while receiving IFN ß for MS. Biopsy showed focal segmental glomerulosclerosis. He went into remisson after cessation of drug and with the aid of angiotensin II antagonists. Here, we report this case and a review of similar cases reported in literature. CONCLUSIONS: Although it's a rare adverse effect and tend to show good prognosis physicians should pay careful attention to symptoms and findings of nephropathy during follow ups of patients under treatment with these agents.

Renal biopsy in the elderly: a single-center experience.

OBJECTIVE: Aging population has been increasing worldwide. So the number of elderly patients presenting with kidney disease has also been increasing. In this retrospective study, we assessed biopsy results of elderly patients and their clinical presentations. MATERIALS AND METHODS: Native renal biopsy results of 98 elderly patients (≥65 years) were analyzed. These 98 patients consisted of 56 males (57.1 %) and 42 females (42.9 %) with a mean age of 70.59 ± 5.31 years (65-88 years). RESULTS: The clinical presentations of our elderly patients were nephritic syndrome (n = 45), acute kidney injury (n = 35), nephrotic syndrome (n = 33), chronic kidney disease (n = 32) and combined nephritic and nephrotic syndrome (n = 14). In patients with nephritic syndrome, the most common diagnosis was crescentic GN (17.8 % type 3 and 13.3 % types 1 and 2). Crescentic GN was also the most frequent among patients with acute kidney injury (37.1 %), while membranous nephropathy was the major histopathological diagnosis in chronic kidney disease patients. In nephrotic syndrome, the most common histopathological diagnosis was AA amyloidosis. None of the patients had a major life-threatening complication. CONCLUSION: Biopsies in the elderly are as safe as in the general population. Renal biopsy should be performed to provide an accurate diagnosis and initiate specific treatment in elderly patients.

Renal involvement in AA amyloidosis: clinical outcomes and survival.

BACKGROUND: The natural history of AA amyloidosis is typically progressive, leading to multiple organ failure and death. We analyzed the etiology as well as clinical and laboratory features of patients with biopsy-proven AA amyloidosis and evaluated the ultimate outcome. METHODS: Seventy-three patients (24 female; mean age 41.85±15.89 years) were analyzed retrospectively. Demographic, clinical and laboratory features were studied and the outcome was assessed. RESULTS: Familial Mediterranean Fever and tuberculosis were the most frequent causes of amyloidosis. Mean serum creatinine and proteinuria at diagnosis were 4.65±4.89 mg/dl and 8.04±6.09 g/day, respectively; and stage I, II, III, IV and V renal disease were present in 19.2%, 13.7%, 16.4%, 11%, and 39.7% of the patients, respectively. ESRD developed in 16 patients during the follow-up period. All of the ESRD patients started a dialysis programme. Thirty patients (41%) died during the follow-up period; median patient survival was 35.9±6.12 months. Old age, tuberculosis etiology, advanced renal disease and low serum albumin levels were associated with a worse prognosis. Serum albumin was a predictor of mortality in logistic regression analysis. CONCLUSION: The ultimate outcome of the patients with AA amyloidosis is poor, possibly due to the late referral to the nephrology clinics. Early referral may be helpful to improve prognosis.

The prevalence of hypertension, valve calcification and left ventricular hypertrophy and geometry in peritoneal dialysis patients.

BACKGROUND/AIMS: Cardiac valve calcification (CVC) and left ventricular (LV) abnormalities are common indicators of a poor prognosis in dialysis patients. We determined the prevalence of hypertension, CVC, LV hypertrophy (LVH) and LV geometry in peritoneal dialysis (PD) patients. METHODS: Eighty-seven patients (50 female; mean age 42 ± 13 years; mean dialysis duration 46 ± 24 months) on strict salt and volume restriction, none of whom were receiving antihypertensives, were included in the study. Blood pressure (BP), biochemical parameters, CVC, LVH and LV geometry were determined. RESULTS: Most patients were normotensive. CVC of the mitral and aortic valves and of both valves were noted in 22, 23 and 15% of patients, respectively. Patients with CVC had significantly higher diastolic BP (p = 0.023), cardiothoracic index (CTI; p = 0.037) and LV mass index (LVMI; p = 0.002). LVH, noted overall in 44% of cases, was present in 62 and 36% of the patients with and without CVC, respectively (p = 0.028). Of the whole group, only 50.6% had normal LV geometry. LVH was associated with lower serum albumin (p = 0.002), higher CTI (p = 0.027) and more frequent CVC (p = 0.028). LVMI was greater in patients with CVC (p = 0.002). CONCLUSION: Strict salt restriction and the achievement of ideal dry weight result in normotension in PD patients. CVC is associated with LVH, both of which are lower in normotensive patients.

Duodenal biopsy for diagnosis of renal involvement in amyloidosis.

Amyloidosis results from extracellular deposition of a fibrillary protein in various organs, and renal biopsy is the best, but a complicated tool for diagnosis. Therefore, alternative biopsy sites have been proposed with varying degrees of sensitivity. We aimed to find the most appropriate biopsy site in patients with chronic kidney disease (CKD) in whom renal biopsy is contraindicated or unavailable. 42 patients (29 male; mean age 46 ± 16 y) with CKD in whom amyloidosis was suspected as the underlying etiology on clinical grounds, but renal biopsy was not available (Group I), and 36 patients (25 male; mean age 40 ± 16 y) with CKD in whom renal biopsy revealed AA-amyloidosis (Group II) were investigated. Upper and lower gastrointestinal tract (GIT) endoscopies were performed and multiple biopsies from gingiva, esophagus, antrum, duodenum and rectum were obtained. In Group I, no amyloidosis was detected in gingival and GIT biopsies among 13 patients. In the remaining 29 patients AA-amyloidosis was detected in various sites with the following frequencies: duodenum 100%, rectum 83%, antrum 79%, esophagus 44% and gingiva 29%. In Group II, frequency of amyloid deposition was 97% in duodenum, 76% each in antrum and rectum, 59% in esophagus and 32% in gingival mucosa. In conclusion, duodenal biopsy is sensitive for diagnosing amyloidosis in CKD patients, and highly correlates with renal amyloidosis.