Immunosuppressive treatment results in patients with primary IgA nephropathy in Turkiye; the data from TSN-GOLD working group.

BACKGROUND: Immunoglobulin A (IgA) nephropathy (IgAN) treatment consists of maximal supportive care and, for high-risk individuals, immunosuppressive treatment (IST). There are conflicting results regarding IST. Therefore, we aimed to investigate IST results among IgAN patients 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 were analyzed. A total of 408 primary IgAN patients treated with IST (65.4% male, mean age 38.4 ± 12.5 years, follow-up 30 (3-218) months) were included and divided into two groups according to treatment protocols (isolated corticosteroid [CS] 70.6% and combined IST 29.4%). Treatment responses, associated factors were analyzed. RESULTS: Remission (66.7% partial, 33.7% complete) was achieved in 74.7% of patients. Baseline systolic blood pressure, mean arterial pressure, and proteinuria levels were lower in responsives. Remission was achieved at significantly higher rates in the CS group (78% vs. 66.7%, p = 0.016). Partial remission was the prominent remission type. The remission rate was significantly higher among patients with segmental sclerosis compared to those without (60.4% vs. 49%, p = 0.047). In the multivariate analysis, MEST-C S1 (HR 1.43, 95% CI 1.08-1.89, p = 0.013), MEST-C T1 (HR 0.68, 95% CI 0.51-0.91, p = 0.008) and combined IST (HR 0.66, 95% CI 0.49-0.91, p = 0.009) were found to be significant regarding remission. CONCLUSION: CS can significantly improve remission in high-risk Turkish IgAN patients, despite the reliance on non-quantitative endpoints for favorable renal outcomes. Key predictors of remission include baseline proteinuria and specific histological markers. It is crucial to carefully weigh the risks and benefits of immunosuppressive therapy for these patients.

The effect of hand fine motor skills on peritoneal dialysis-related peritonitis.

INTRODUCTION: We aimed to evaluate the relationship between the hand fine motor skills of peritoneal dialysis (PD) practitioners and PD-related peritonitis. METHODS: This multicenter prospective observational study was conducted with 120 incident PD patients. Patients were divided into two groups who had PD-related peritonitis within the first year as Group 1, and those who did not as Group 2. Hand fine motor skills were evaluated by Nine-Hole Peg Test (NHPT) and Nut Screwing Test (NST). RESULTS: Initial NHPT (28.5 ± 6.0 s vs. 25.8 ± 5.0 s, p = 0.011) and NST (82.3 (61.5-102.8) s versus 65.3 (52.3-88.5) s p = 0.023) scores were significantly higher in Group 1 than Group 2. In multivariate logistic regression analysis, NHPT, Body Mass Index, Mini-Mental Test, self PD practitioner, and catheter complications were found to be independent variables in predicting PD-related peritonitis. CONCLUSION: Decreased hand fine motor skills of PD patients is a risk factor for peritonitis.

The perception of nephrologists about peritoneal dialysis in Turkey.

BACKGROUND: We aimed to evaluate the perceptions of nephrologists about peritoneal dialysis (PD) in Turkey. MATERIALS AND METHODS: An anonymous survey was conducted to a total of 150 nephrologists. Demographic data, perceptions, PD indications, contraindications, and possible reasons for low preference of PD were questioned. RESULTS: In decision making, 93.4% of all stated that patients prioritized the doctor's opinion and 80.7% considered PD is the best initial dialysis option. The presence of many HD facilities (70.7%), inadequate knowledge, and education of patients (70%), physicians' reluctance to practice PD (70%), unwillingness of patients (68.7%), negative effect of other patients (67.3%), inadequate experience of PD staff (58.7%), and nonencouragement of PD by the state (58.7%) were the leading reasons of low prevalence. CONCLUSION: Implementation of comprehensive predialysis education programs for patients, informing government and hospital officials about PD advantages, and reinforcing PD principles to the nephrologists could improve the low prevalence of PD in Turkey.

Modified histopathological classification with age-related glomerulosclerosis for predicting kidney survival in ANCA-associated glomerulonephritis.

BACKGROUND: The histopathological classification of ANCA-GN divides patients into four groups based on signs of glomerular injury. However, this classification did not consider age-related glomerulosclerosis. In this study, we aimed to compare the prediction of renal survival between Berden's ANCA-GN histopathological classification and ANCA-GN histopathological classification modified with age-related glomerulosclerosis. METHODS: Between January 2004 and December 2019, 65 patients diagnosed with ANCA-GN were enrolled. Demographic, laboratory, and histopathologic findings were retrospectively analyzed. Renal survival analyses were compared according to classical and modified ANCA-GN histopathological classifications. Multivariate Cox regression analysis for the factors affecting renal survival was performed. RESULTS: In Berden's ANCA-GN histopathological classification, 15 patients were in the focal group, 21 in the crescentic, 21 in the sclerotic, and 8 in the mixed group. The ANCA-GN histopathological classification model generated statistically significant predictions for renal survival (p = 0.022). When the histopathological classification was modified with age-related glomerulosclerosis, eight of the nine patients previously classified in the sclerotic group were classified in the mixed and one in the crescentic groups. Modification of histopathological classification with age-related glomerulosclerosis increases the statistical significance in renal survival analysis (p = 0.009). The multivariate Cox regression analysis showed that the disease-related global sclerotic glomeruli percentage and serum creatinine level were significant independent factors. CONCLUSION: Modification of Berden's ANCA-GN histopathological classification model with age-related glomerulosclerosis may increase the statistical significance of the histopathological classification model.

Evaluation of Outcomes of Peritoneal Dialysis Patients in the Post-COVID-19 Period: A National Multicenter Case-Control Study from Turkey.

INTRODUCTION: There are not enough data on the post-CO-VID-19 period for peritoneal dialysis (PD) patients affected from COVID-19. We aimed to compare the clinical and laboratory data of PD patients after COVID-19 with a control PD group. METHODS: This study, supported by the Turkish Society of Nephrology, is a national, multicenter retrospective case-control study involving adult PD patients with confirmed COVID-19, using data collected from April 21, 2021, to June 11, 2021. A control PD group was also formed from each PD unit, from patients with similar characteristics but without COVID-19. Patients in the active period of COVID-19 were not included. Data at the end of the first month and within the first 90 days, as well as other outcomes, including mortality, were investigated. RESULTS: A total of 223 patients (COVID-19 group: 113, control group: 110) from 27 centers were included. The duration of PD in both groups was similar (median [IQR]: 3.0 [1.88-6.0] years and 3.0 [2.0-5.6]), but the patient age in the COVID-19 group was lower than that in the control group (50 [IQR: 40-57] years and 56 [IQR: 46-64] years, p < 0.001). PD characteristics and baseline laboratory data were similar in both groups, except serum albumin and hemoglobin levels on day 28, which were significantly lower in the COVID-19 group. In the COVID-19 group, respiratory symptoms, rehospitalization, lower respiratory tract infection, change in PD modality, UF failure, and hypervolemia were significantly higher on the 28th day. There was no significant difference in laboratory parameters at day 90. Only 1 (0.9%) patient in the COVID-19 group died within 90 days. There was no death in the control group. Respiratory symptoms, malnutrition, and hypervolemia were significantly higher at day 90 in the COVID-19 group. CONCLUSION: Mortality in the first 90 days after COVID-19 in PD patients with COVID-19 was not different from the control PD group. However, some patients continued to experience significant problems, especially respiratory system symptoms, malnutrition, and hypervolemia.

The effect of calcineurin inhibitors on anthropometric measurements in kidney transplant recipients.

BACKGROUND: This study was designed to investigate the effect of calcineurin inhibitors (CNIs), cyclosporine (CsA), and tacrolimus (Tac) on anthropometrics in kidney transplant recipients. METHODS: 111 of 128 adult kidney transplant recipients who received post-transplant CNIs were included in this retrospective study. Anthropometrics were recorded in the pre-transplant and post-transplant 4-year follow-up periods (1st, 3rd, 6th, 12th, 24th, 36th and 48th months). RESULTS: Compared to pre-transplant values, significant increases in body weight and body mass index (between 3rd and 48th months), waist and hip circumferences (between 1st and 48th months), waist-to-hip ratio (between 1st and 3rd or 6th months) and neck circumference (between 1st and 12th or 24th months) were observed in both CsA and Tac groups. A significant increase was noted in post-transplant body fat percentage values for the 3rd to 24th months in the CsA group, whereas for the 24th to 48th months in both CsA and Tac groups. Hip circumferences percentage changes from the pre-transplant period to the 1st, 12th and 24th months were significantly higher in CsA than in the Tac group. At each time point, there was no significant difference in percentage changes for other anthropometric parameters between the CsA and Tac groups. De novo diabetes mellitus developed in 8.3% of the CsA group and 19.1% of the Tac group. CONCLUSIONS: After a successful kidney transplant, anthropometric measurements increase in most recipients. Although the effect of calcineurin inhibitor type on weight gain is unclear, a regression analysis showed that CNI type was not a risk factor for the development of obesity in the 48th month. However, it is helpful to be cautious about its dyslipidemic effect in patients using CsA and the potential hazards of using Tac in patients with a diabetic predisposition.

Characteristics and Survival Results of Peritoneal Dialysis Patients Suffering from COVID-19 in Turkey: A Multicenter National Study.

INTRODUCTION: We aimed to study the characteristics of peritoneal dialysis (PD) patients with coronavirus disease-19 (COVID-19), determine the short-term mortality and other medical complications, and delineate the factors associated with COVID-19 outcome. METHODS: In this multicenter national study, we included PD patients with confirmed COVID-19 from 27 centers. The baseline demographic, clinical, laboratory, and radiological data and outcomes at the end of the first month were recorded. RESULTS: We enrolled 142 COVID-19 patients (median age: 52 years). 58.2% of patients had mild disease at diagnosis. Lung involvement was detected in 60.8% of patients. Eighty-three (58.4%) patients were hospitalized, 31 (21.8%) patients were admitted to intensive care unit and 24 needed mechanical ventilation. Fifteen (10.5%) patients were switched to hemodialysis and hemodiafiltration was performed for four (2.8%) patients. Persisting pulmonary symptoms (n = 27), lower respiratory system infection (n = 12), rehospitalization for any reason (n = 24), malnutrition (n = 6), hypervolemia (n = 13), peritonitis (n = 7), ultrafiltration failure (n = 7), and in PD modality change (n = 8) were reported in survivors. Twenty-six patients (18.31%) died in the first month of diagnosis. The non-survivor group was older, comorbidities were more prevalent. Fever, dyspnea, cough, serious-vital disease at presentation, bilateral pulmonary involvement, and pleural effusion were more frequent among non-survivors. Age (OR: 1.102; 95% CI: 1.032-1.117; p: 0.004), moderate-severe clinical disease at presentation (OR: 26.825; 95% CI: 4.578-157.172; p < 0.001), and baseline CRP (OR: 1.008; 95% CI; 1,000-1.016; p: 0.040) were associated with first-month mortality in multivariate analysis. DISCUSSION/CONCLUSIONS: Early mortality rate and medical complications are quite high in PD patients with COVID-19. Age, clinical severity of COVID-19, and baseline CRP level are the independent parameters associated with mortality.

Clinical and pathologic features of primary membranous nephropathy in Turkey: a multicenter study by the Turkish Society of Nephrology Glomerular Diseases Working Group.

BACKGROUND: We aimed to evaluate the features of primary membranous nephropathy (MNP) in Turkish people. METHODS: This is a retrospective analysis of patients with biopsy-proven primary MNP. We obtained the data collected between 2009 and 2019 in the primary glomerulonephritis registry of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD). Patients with a secondary cause for MNP were excluded. Clinical, demographic, laboratory, and histopathological findings were analyzed. RESULTS: A total of 995 patients with primary MNP were included in the analyses. Males constituted the majority (58.8%). The mean age was 48.4 ± 13.9 years. The most common presentation was the presence of nephrotic syndrome (81.7%) and sub nephrotic proteinuria (10.3%). Microscopic hematuria was detected in one-third of patients. The median estimated glomerular filtration rate (eGFR) was 100.6 mL/min/1.73 m2 (IQR, 75.4-116.3), and median proteinuria was 6000 mg/d (IQR, 3656-9457). Serum C3 and C4 complement levels were decreased in 3.7 and 1.7% of patients, respectively. Twenty-four (2.4%) patients had glomerular crescents in their kidney biopsy samples. Basal membrane thickening was detected in 93.8% of cases under light microscopy. Mesangial proliferation and interstitial inflammation were evident in 32.8 and 55.9% of the patients, respectively. The most commonly detected depositions were IgG (93%), C3 complement (68.8%), and kappa and lambda immunoglobulin light chains (70%). Although renal functions were normal at presentation, vascular, interstitial, and glomerular findings were more prominent on biopsy in hypertensive patients. No significant effect of BMI on biopsy findings was observed. CONCLUSIONS: Despite some atypical findings, the main features of primary MNP in Turkey were similar to the published literature. This is the largest MNP study to date conducted in Turkish people.

The perspectives of dialysis patients about the Covid-19 pandemic and differences between the modalities.

The novel coronavirus disease was declared as a pandemic and CKD is an important risk factor for morbidity and mortality. Dialysis has additional contributions on transmission risk so prompt preventive strategies were implemented for dialysis patients. We aimed to evaluate pandemic-related perceptions and concerns of dialysis patients and differences between dialysis modalities. An anonymous survey for assessing concerns, knowledge, and attitudes about the pandemic was sent online to a total of 339 patients on maintenance dialysis at four tertiary dialysis centers in Turkey. A total of 309 patients (54.9 ± 15.1 years, 51.6% females, 55.7% in-center HD, 44.3% peritoneal dialysis) enrolled. The anonymous online survey was conducted at the end of April 2020. HD patients were more concerned about transmission risk (p = 0.002) and risks associated with the dialysis treatment environment and the transport methods (p < 0.001). The total concern score was significantly higher in the HD group (2.60 ± 0.93 vs. 1.65 ± 0.54, p < 0.001). The knowledge about the pandemic and prevention methods and the attitudes of prevention were similar between the groups (p = 0.161 and 0.418, respectively). The compliance rate of personal preventive strategies was 98.1%. Considering changing the current dialysis modality due to the pandemic was higher in the HD group (p < 0.001). Although the preventive strategies were performed properly in the HD centers, HD patients were more concerned about the Covid-19 outbreak compared with PD. Our results support home dialysis treatments for modality decisions with patients' positive perspective of PD over HD during the pandemic.

The Effect of Smoking on Endothelial Dysfunction in Autosomal Dominant Polycystic Kidney Disease Patients with Preserved Renal Function.

BACKGROUND: In autosomal dominant polycystic kidney disease (ADPKD), endothelial dysfunction (ED) is common and occurs much earlier than kidney function impairment. The impact of smoking on ED in ADPKD patients has not been previously studied. The aim of this study was to investigate the potential contribution of smoking habits to ED and subclinical atherosclerosis in these patients. METHODS: This case-control study included 54 ADPKD patients with preserved renal function and 45 healthy control subjects. ED was assessed using ischemia-induced forearm flow-mediated dilatation (FMD). Carotid intima-media thickness (CIMT) was measured from 10 mm proximal to the right common carotid artery. Clinical demographic characteristics and laboratory data were recorded for the patients and control group. Regression analysis was used to determine independent associations of ED and CIMT. RESULTS: FMD was significantly lower in the ADPKD patients (19.5 ± 5.63 vs. 16.56 ± 6.41, p = .018). Compared with nonsmoker ADPKD patients, smoker patients had significantly lower FMD values (18.19 ± 6.52 vs. 13.79 ± 5.27, p = .013). In multiple regression analysis, age (ß = -0.294, 95% CI: -0.392: -1.96, p = .001) for FMD and smoking (ß = 1.328, 95% CI: 0.251, 2.404, p = .017) for CIMT were independent predictors. CONCLUSIONS: Patients with ADPKD had more impaired endothelial function and subclinical atherosclerosis compared with control subjects. Smoking may increase the risk of subclinical atherosclerosis in ADPKD patients.

Morning blood pressure surge in early autosomal dominant polycystic kidney disease and its relation with left ventricular hypertrophy.

INTRODUCTION: The activation of the sympathetic nervous system, which usually leads to a swift surge in blood pressure in the morning hours (MBPS) may be the cause of left ventricular hypertrophy (LVH) and endothelial dysfunction (ED) in early autosomal dominant polycystic kidney disease (ADPKD) patients. We studied the association between MBPS and LVH in ADPKD patients with preserved renal functions. METHODS: Patients with ADPKD with preserved renal functions were enrolled. Prewaking MBPS was calculated using ambulatory blood pressure monitoring. The patients were categorized as MBPS (≥median) and non-MBPS (

Effect of Parathyroidectomy Timing in Kidney Transplant Recipients on Graft Function.

OBJECTIVES: Persistent hyperparathyroidism can have a deleterious effect on graft function in kidney transplant recipients, although serum calcium, phosphorus, and parathyroid hormone levels tend to normalize after successful transplant. Parathyroidectomy can result in sustained amelioration of persistent hyperparathyroidism despite graft failure risk and unfavorable graft outcomes. Data on this issue are limited and conflicting. Here, we evaluated the effects of parathyroidectomy on graft function in kidney transplant recipients. MATERIALS AND METHODS: This retrospective study included 249 adult kidney transplant recipients (121 deceased-donor/128 living-donor; 142 males/107 females; mean age of 39.3 ± 11.6 y; mean follow-up of 46.5 ± 23.5 mo). Participants were grouped as those without (n = 222), those with pretransplant (n = 12), and those with posttransplant (n = 15) parathyroidectomy. Graft outcomes and serum calcium, phosphorus, and parathyroid hormone levels were studied. RESULTS: Serum calcium levels at baseline and at 1, 3, 6, and 12 months and parathyroid hormone levels at baseline and at 6 and 12 months were higher and serum phosphorus levels at 3, 6, and 12 months were lower in the posttransplant parathyroidectomy group versus the other groups (P < .001). We observed no significant differences between groups regarding serum calcium, phosphorus, and parathyroid hormone levels at last visit. Estimated glomerular filtration rates at 3, 6, and 12 months and at last visit in the pretransplant parathyroidectomy group were higher than in those without parathyroidectomy (P < .05) and higher at 6 and 12 months than in the posttransplant parathyroidectomy group (P < .05). No significant differences regarding graft loss and patient mortality were observed among the 3 groups (P > .05). CONCLUSIONS: Parathyroidectomy resulted in sustained decreased levels of serum calcium and parathyroid hormone. We observed no graft failure risk associated with parathyroidectomy in our study. Parathyroidectomy before transplant is advantageous with better graft function.

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.

Mortality analysis of COVID-19 infection in chronic kidney disease, haemodialysis and renal transplant patients compared with patients without kidney disease: a nationwide analysis from Turkey.

BACKGROUND: Chronic kidney disease (CKD) and immunosuppression, such as in renal transplantation (RT), stand as one of the established potential risk factors for severe coronavirus disease 2019 (COVID-19). Case morbidity and mortality rates for any type of infection have always been much higher in CKD, haemodialysis (HD) and RT patients than in the general population. A large study comparing COVID-19 outcome in moderate to advanced CKD (Stages 3-5), HD and RT patients with a control group of patients is still lacking. METHODS: We conducted a multicentre, retrospective, observational study, involving hospitalized adult patients with COVID-19 from 47 centres in Turkey. Patients with CKD Stages 3-5, chronic HD and RT were compared with patients who had COVID-19 but no kidney disease. Demographics, comorbidities, medications, laboratory tests, COVID-19 treatments and outcome [in-hospital mortality and combined in-hospital outcome mortality or admission to the intensive care unit (ICU)] were compared. RESULTS: A total of 1210 patients were included [median age, 61 (quartile 1-quartile 3 48-71) years, female 551 (45.5%)] composed of four groups: control (n = 450), HD (n = 390), RT (n = 81) and CKD (n = 289). The ICU admission rate was 266/1210 (22.0%). A total of 172/1210 (14.2%) patients died. The ICU admission and in-hospital mortality rates in the CKD group [114/289 (39.4%); 95% confidence interval (CI) 33.9-45.2; and 82/289 (28.4%); 95% CI 23.9-34.5)] were significantly higher than the other groups: HD = 99/390 (25.4%; 95% CI 21.3-29.9; P < 0.001) and 63/390 (16.2%; 95% CI 13.0-20.4; P < 0.001); RT = 17/81 (21.0%; 95% CI 13.2-30.8; P = 0.002) and 9/81 (11.1%; 95% CI 5.7-19.5; P = 0.001); and control = 36/450 (8.0%; 95% CI 5.8-10.8; P < 0.001) and 18/450 (4%; 95% CI 2.5-6.2; P < 0.001). Adjusted mortality and adjusted combined outcomes in CKD group and HD groups were significantly higher than the control group [hazard ratio (HR) (95% CI) CKD: 2.88 (1.52-5.44); P = 0.001; 2.44 (1.35-4.40); P = 0.003; HD: 2.32 (1.21-4.46); P = 0.011; 2.25 (1.23-4.12); P = 0.008), respectively], but these were not significantly different in the RT from in the control group [HR (95% CI) 1.89 (0.76-4.72); P = 0.169; 1.87 (0.81-4.28); P = 0.138, respectively]. CONCLUSIONS: Hospitalized COVID-19 patients with CKDs, including Stages 3-5 CKD, HD and RT, have significantly higher mortality than patients without kidney disease. Stages 3-5 CKD patients have an in-hospital mortality rate as much as HD patients, which may be in part because of similar age and comorbidity burden. We were unable to assess if RT patients were or were not at increased risk for in-hospital mortality because of the relatively small sample size of the RT patients in this study.

The Comparison Spondin 2 Levels in Primary Glomerular Diseases.

Spondin 2 (SPON2) plays an important role in multiple processes and is a member of the Spondin 2/F-spondin family of extracellular matrix proteins. We investigated serum SPON2 levels and its correlation with renal functions and urine protein excretion in different glomerular diseases. The cohort included 97 consecutive adults with persistant proteinuria (>300 mg/day) with the diagnosis of focal segmental glomerulosclerosis (FSGS), membranous glomerulonephritis (MN), IgA nephropathy (IgAN), membranoproliferative glomerulonephritis (MPGN), and AA amyloidosis and the control groups with 15 polycystic kidney disease (PKD) and 32 healthy people. Serum SPON2 levels in MN (64.6 ng/mL), FSGS (47.8 ng/mL), IgAN (52.6 ng/mL), MPGN (54.6 ng/mL), and AA amyloidosis (60.7 ng/mL) groups were higher than those of the control (26.4 ng/mL) and nonglomerular disease groups (PKD) (15.3 ng/mL). Only serum SPON2 levels were correlated with serum uric acid and triglyceride levels in patients with glomerular disease. This is the first study to show that serum SPON2 levels are similar in different glomerular diseases and that there is no correlation between SPON2 and proteinuria grade.

Hepatotoxicity associated with eculizumab in a patient with atypical hemolytic uremic syndrome / Hepatotoxicidad asociada con eculizumab en un paciente con síndrome urémico hemolítico atípico

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Clinical outcomes and survival in AA amyloidosis patients / Desfechos clínicos e sobrevida em pacientes com amiloidose AA

Abstract Aim Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. Methods: A total of 81 patients (51 males, 30 females) with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results. Results: Most of the patients (55.6%) had nephrotic range proteinuria at diagnosis. Most frequent underlying disorders were familial Mediterranean fever (21.2%) and rheumatoid arthritis (10.6%) in the good outcome group and malignancy (20%) in the poor outcome group. Only diastolic blood pressure in the good outcome group and phosphorus level in the poor outcome group was higher. Serum creatinine levels increased after treatment in both groups, while proteinuria in the good outcome group decreased. Increase in serum creatinine and decrease in estimated glomerular filtration rate of the poor outcome group were more significant in the good outcome group. At the time of diagnosis 18.5% and 27.2% of all patients had advanced chronic kidney disease (stage 4 and 5, respectively). Median duration of renal survival was 65 ± 3.54 months. Among all patients, 27.1% were started dialysis treatment during the follow-up period and 7.4% of all patients underwent kidney transplantation. Higher levels of systolic blood pressure [hazard ratios 1.03, 95% confidence interval: 1-1.06, p = 0.036], serum creatinine (hazard ratios 1.25, 95% confidence interval: 1.07-1.46, p = 0.006) and urinary protein excretion (hazard ratios 1.08, 95% confidence interval: 1.01-1.16, p = 0.027) were predictors of end-stage renal disease. Median survival of patients with organ involvement was 50.3 ± 16 months. Conclusion Our study indicated that familial Mediterranean fever constituted a large proportion of cases and increased number of patients with idiopathic amyloid A amyloidosis. Additionally, it was observed that patient survival was not affected by different etiological causes in amyloid A amyloidosis.

Resumo Objetivo: A amiloidose AA é uma complicação rara de condições inflamatórias crônicas. A maior parte dos pacientes com amiloidose AA apresenta nefropatia, que leva à insuficiência renal e à morte. Estudaram-se as características clínicas e a sobrevida em pacientes com amiloidose AA. Métodos: Analisaram-se retrospectivamente 81 pacientes (51 homens, 30 mulheres) com amiloidose AA comprovada por biópsia renal. Os pacientes foram divididos em grupos de desfecho bom e ruim de acordo com os resultados de sobrevida. Resultados: A maior parte dos pacientes (55,6%) tinha proteinúria na faixa nefrótica no momento do diagnóstico. Os distúrbios subjacentes mais frequentes foram a febre familiar do Mediterrâneo (FFM, 21,2%) e a artrite reumatoide (10,6%) no grupo de desfecho bom e a malignidade (20%) no grupo de desfecho ruim. Somente a pressão arterial diastólica no grupo de desfecho bom e o nível de fósforo no grupo de desfecho ruim foram mais elevados. Os níveis séricos de creatinina aumentaram após o tratamento em ambos os grupos, enquanto a proteinúria diminuiu no grupo de desfecho bom. O aumento na creatinina sérica e a diminuição na TFGe do grupo de desfecho ruim foram mais significativos no grupo de desfecho bom. No momento do diagnóstico, 18,5% e 27,2% de todos os pacientes tinham doença renal crônica avançada (estágios 4 e 5, respectivamente). A duração média da sobrevida renal foi de 65 ± 3,54 meses. Entre todos os pacientes, 27,1% iniciaram tratamento de diálise durante o período de seguimento e 7,4% de todos os pacientes foram submetidos a transplante renal. Níveis elevados de pressão arterial sistólica [taxas de risco (HR) 1,03, intervalo de confiança (IC) de 95%: 1 a 1,06, p = 0,036], creatinina sérica (HR 1,25, IC 95%: 1,07 a 1,46, p = 0,006) e excreção urinária de proteínas (HR 1,08, IC 95%: 1,01 a 1,16, p = 0,027) foram preditores de doença renal terminal. A mediana da sobrevida de pacientes com comprometimento de órgãos foi de 50,3 ± 16 meses. Conclusão: O presente estudo indicou que a FFM constituiu uma grande proporção de casos e crescente quantidade de pacientes com amiloidose AA idiopática. Adicionalmente, observou-se que a sobrevida do paciente não foi afetada pelas diferentes causas etiológicas na amiloidose AA.

Clinical outcomes and survival in AA amyloidosis patients.

AIM: Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. METHODS: A total of 81 patients (51 males, 30 females) with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results. RESULTS: Most of the patients (55.6%) had nephrotic range proteinuria at diagnosis. Most frequent underlying disorders were familial Mediterranean fever (21.2%) and rheumatoid arthritis (10.6%) in the good outcome group and malignancy (20%) in the poor outcome group. Only diastolic blood pressure in the good outcome group and phosphorus level in the poor outcome group was higher. Serum creatinine levels increased after treatment in both groups, while proteinuria in the good outcome group decreased. Increase in serum creatinine and decrease in estimated glomerular filtration rate of the poor outcome group were more significant in the good outcome group. At the time of diagnosis 18.5% and 27.2% of all patients had advanced chronic kidney disease (stage 4 and 5, respectively). Median duration of renal survival was 65±3.54 months. Among all patients, 27.1% were started dialysis treatment during the follow-up period and 7.4% of all patients underwent kidney transplantation. Higher levels of systolic blood pressure [hazard ratios 1.03, 95% confidence interval: 1-1.06, p=0.036], serum creatinine (hazard ratios 1.25, 95% confidence interval: 1.07-1.46, p=0.006) and urinary protein excretion (hazard ratios 1.08, 95% confidence interval: 1.01-1.16, p=0.027) were predictors of end-stage renal disease. Median survival of patients with organ involvement was 50.3±16 months. CONCLUSION: Our study indicated that familial Mediterranean fever constituted a large proportion of cases and increased number of patients with idiopathic amyloid A amyloidosis. Additionally, it was observed that patient survival was not affected by different etiological causes in amyloid A amyloidosis.