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BACKGROUND AND AIMS To explore the frequency and impact of an autoimmune disease past-medical history (PMH) in the clinical picture and outcomes of patients with ANCA-associated vasculitis (AAV). METHOD This is a retrospective study of patients with biopsy-proven AAV, >16 years old, with detailed information about their PMH. Outcomes of interest included remission, treatment resistance, relapse, end-stage kidney disease (ESKD), and death. RESULTS 215 patients with biopsy-proven AAV and available information regarding their PMH were studied. A total of 65 (30.2%) of them had a history of autoimmune disease prior to PIV diagnosis. The mean age overall was 53.9 years. One hundred and five patients (48.8%) were positive for PR3-ANCA, 101(47.2%) for MPO-ANCA, and 9(4.2%) were negative ANCA. Granulomatosis with polyangiitis was diagnosed in 79 (36.7%), microscopic polyangiitis in 101(47.0%) and renal-limited vasculitis in 35 (16.3%) individuals. Remission rate was similar among patients with and without a PMH of autoimmune disease. Time-to-event analysis indicated that the relapse-free survival was significantly longer in patients with PMH of autoimmune disease (148.2 versus 52.8 months, P-value < .001). After adjusting for covariates, autoimmune disease history was associated with significantly lower risk of relapse {HR 0.31, [95% confidence interval (CI) 0.14–0.69]}, which remained significant in males, patients ≥ 60 years old and those with C/PR3-ANCA, kidney and lung involvement. CONCLUSION Patients with a PMH of autoimmune disease, prior to AAV diagnosis, experienced significantly fewer relapses after achievement of remission, compared with patients without such a history, underlining the importance of individualization of maintenance immunosuppressive therapy, given the different etiopathogenetic settings the disease was developed.

BACKGROUND AND AIMS Hypertension is the most prevalent cardiovascular risk factor in kidney transplant recipients (KTRs). Preliminary data suggest similar ambulatory blood pressure (ΒP) levels in KTRs and hemodialysis (HD) patients. This is the first study evaluating in comparison the full ambulatory BP profile and short-term BP variability (BPV) in KTRs versus HD patients. METHOD Two hundred four KTRs were matched (2:1 ratio) with 102 HD patients for age and gender. BP levels, BP trajectories and BPV indices over a 24-h ambulatory BP monitoring (ABPM) in KTRs were compared against both the first and second 24-h period of a standard 48-h ABPM in HD. To evaluate the effect of renal replacement treatment and time on ambulatory BP levels, two-way-ANOVA for repeated-measurements was performed. RESULTS KTRs had significantly lower SBP and pulse-pressure (PP) levels compared with HD during all periods studied (24-h SBP: KTR: 126.5 ± 12.1 mmHg; HD first 24-h: 132.0 ± 18.1 mmHg, P = 0.006; second 24-h: 134.3 ± 17.7 mmHg, P CONCLUSION SBP and PP levels and trajectories, and BPV were significantly lower in KTRs compared to age- and gender-matched HD patients during all periods studied. These findings suggest a more favorable ambulatory BP profile in KTRs, in contrast with previous observations.

Background and Aims Focal Segmental Glomerulosclerosis (FSGS) recurrence after kidney transplantation (KTx) is relatively frequent and is associated with poor graft survival. The aim of this study was to investigate which management strategies were associated with better outcomes in our cohort of KTx recipients with primary FSGS. Method We retrospectively collected data on patients with primary FSGS who received a KTx between 1993 and 2019. A history of biopsy proven FSGS in native kidneys and new onset of significant proteinuria early post-KTx led to the diagnosis of recurrence, which was confirmed by graft biopsy. Results From 1993 to 2019 we performed 46 KTxs in patients with primary FSGS. We identified 26 episodes of recurrence in 25 patients, 67% of them occurring in males. They were younger at the time of KTx (33.8 vs 41.1 years old, p=0.067) and had progressed to end stage renal disease (ESRD) faster after FSGS diagnosis (61.4 vs 111.2 months, p=0.038), while they were less likely to have received prophylactic plasmapheresis (61.5% vs 90%, p=0.029). 76.7% of recurrences were found early, after a median of 0.5 months (IQR 0.1-1) with a median proteinuria was 8.5 (IQR 4.9-11.9) g/day. All patients with recurrence were treated with plasmapheresis, while 8 (30.7%) additionally received rituximab, 1 (3.8%) abatacept and 4 (15.4%) ACTH. 7 (27%) patients experienced complete and 11 (42.3%) partial remission after a mean time of 3 (±1.79) and 4.4 (±2.25) months respectively. Prognosis was worse for patients who experienced a recurrence. 11 (42.3%) patients lost their graft from FSGS in a median time of 33 (IQR 17.5-43.3) months. Conclusion In this series of patients, primary FSGS recurred frequently after KTx. Prophylacic plasmapheresis was shown efficacious in avoiding FSGS recurrence, while timely diagnosis and plasmapheresis-based regimens induced remission in more than half of the patients.

Background and Aims Cardiovascular disease is highly prevalent in the chronic kidney disease population and associates with higher morbidity and mortality even in young ages. The aim of the present study was to assess data from 24h ambulatory blood pressure monitoring (ABPM) and 24h pulse wave analysis (PWA) in dialyzed children and investigate their association with left ventricular mass as a marker of increased cardiovascular risk. Method Children and adolescents aged 6 to 18 years, on dialysis for more than 12 months, [16 on peritoneal dialysis (PD) and 12 on hemodialysis (HD)], underwent 24h ABPM, 24h PWA, and echocardiography. Results Peripheral and central blood pressures (BPs), mean pulse wave velocity (PWV) and left ventricular mass index (LVMI) were similar between PD and HD patients (figure 1a,b). Among PWA hemodynamic parameters, augmentation index (AIx75), cardiac index (CI), and heart rate were higher in the HD group (p Conclusion HD pediatric patients demonstrate higher AIx75 and CI compared to PD patients even in the absence of LVH. These results suggest distinct patterns of vascular and cardiac functional changes in HD patients that may increase cardiovascular risk beyond LVH.

Background and Aims Hyperkalaemia is a frequent and potentially life-threatening condition in patients with CKD. Even after successful kidney transplantation, kidney transplant recipients have mild to severe CKD. Moreover, they share co-morbid conditions and frequently use medications that predispose to hyperkalaemia. This study aimed to examine the prevalence and factors associated with hyperkalaemia in this population. Method Over a pre-specified period of 6 months (September 1st 2019 to March 31st 2020), we recorded in a cross-sectional fashion information on serum potassium and relevant demographics, co-morbidities, medications, laboratory and transplant-associated variables in clinically stable kidney transplant recipients attending the Transplant Outpatient Clinic of our Department. Ηyperkalaemia was classified as follows: serum potassium level >5 mEq/L or >5 with concomitant use of sodium polystyrene sulfonate; ≥5.2; and ≥5.5 mEq/L. Univariate and multiple logistic regression analyses were used to identify factors associated with increased serum potassium >5.0 mEq/L. Results The study population consisted of 582 stable kidney transplant recipients, 369 (63.4%) males, aged 52.4±13.5 years, with eGFR of 55.8±20.1 ml/min/1.73m2 transplanted for more than one year. The prevalence of hyperkalaemia defined as potassium >5, >5 and use of sodium polystyrene sulfonate, ≥ 5.2 or ≥5.5, was 22.7%, 22.7%, 14.4% and 4.1% (132, 132, 84 and 24 patients), respectively. In multivariate analysis, male gender (OR 2.020, 95% CI 1.264-3.227) and use of renin-angiotensin-aldosterone-system blockers (OR 1.628, 95% CI 1.045-2.536), were independently associated with hyperkalaemia, while higher eGFR (OR 0.967, 95% CI 0.955-0.979) and diuretic use (OR 0.140, 95% CI 0.046-0.430) were associated with lower odds of the disorder. Conclusion The prevalence of mild hyperkalaemia in stable kidney transplant recipients is common but that of moderate or severe hyperkalemia is relatively uncommon. Male gender and low eGFR are the most potent factors associated with hyperkalaemia. Among a wide range of factors only male gender, RAAS blockade and low eGFR increased the odds of hyperkalemia.

Background and Aims Severe kidney involvement represents a frequent clinical scenario for patients with pauci-immune (PI) vasculitis. We aimed to explore the factors, which are associated with prognosis, in short and long term, following administration of appropriate immunosuppressive therapy. Method Patients were included if they had biopsy proven PI glomerulonephritis (GN) with estimated GFR20 ml/min/1.73 m2 with no signs of vasculitis. Histopathological evaluation included arteriosclerosis, % of normal glomeruli, activity index, chronicity index. Results A total of 83 patients, with a mean age of 59.6 (15.05) years were included. There were, 45 males (54.2%). After 3 months, 59 patients (71.1%) had responded to immunosuppressive therapy, 16 (19.3%) were dialysis depended, 5(6.02%) died and 2 were lost in follow up. By the end of the 1st year, 59 patients (71.1%) achieved remission, 16(19.3%) ended up in ESKD and 6(7.4%) died. Factors which were associated with treatment response included MPO-ANCA positivity [odds ratio OR:3.9, 95%CI (1.13-13.37) p=0.03], eGFR>10ml/min/1.73m2 at presentation [OR:2.5, 95% CI(0.86-7.30), p=0.009], normal glomeruli >10% [OR:3.8, 95%CI (1.24-12.1), p=0.02], and chronicity index more than 6 [OR:6.2, 95% CI(1.77-22.4), p=0.004]. Risk factors associated with ESKD included non-response to immunosuppressive therapy [Relative Risk RR:0.05, 95%CI (0.01-0.2) p75 years [RR:3.2, 95% CI (0.9-10.6) p=0.055]. Two of the 6 deaths were disease related. Conclusion A significant proportion of patients with PI-GN, who presented with severe renal dysfunction, responded to immunosuppressive therapy and recovered renal function approximately 3 months after initiation of therapy. The most important risk factors for ESKD were age>75 years

Background and Aims Advances in immunosuppressants, used for kidney transplantation (KTx) have significantly improved outcomes in this field. We aimed to compare the long-term results of KTx in recipients with IgAN, as cause of end stage kidney disease (ESKD), with those of patients, with non-glomerular causes of renal failure, during the modern era of immunosuppression for KTx. Method This is a retrospective, case control study, in which were included patients who received a KTx after 2000. Patients were eligible to be included, if they had IgAN in a native kidney biopsy specimen and follow up longer than 1 year after KTx. IgAN patients were compared to a control-group of patients with non-glomerular primary causes of ESKD matched for age, gender, date of KTx and donor source. Graft biopsies were performed by clinical indication. Patients with ABO incompatible KTx, preemptive KTx, re-KTx, PRA>50%, major surgical complications during the 1st post-KTx month or non-compliance were excluded from the analysis of outcome. Primary outcomes of interest included graft function, patient and graft survival at end of follow up. The rate of IgAN recurrence in the graft and its impact in renal function and survival were also recorded. Computed GFR was calculated using the Modification of Diet in Renal Disease formula. Results A total of 102 KTx recipients with biopsy-proven IgAN, were compared to 204 controls with non-glomerular causes of ESKD. The mean age of patients with IgAN was 43.2(±10.15) years and 68(66.7%) were men. The mean time in dialysis was 50.2(±48.7) months and 59(57.8%) of them received a graft from a living donor. The mean cold ischemia time was 17.5(±6.7) hours, while 22(21.6%) of patients experienced delayed graft function. 97.05% of patients received induction therapy with an anti-CD25 inhibitor and 99.3% were maintained with a triple immunosuppressive regimen consisted of a mycophenolate mofetil formulation, a calcineurin inhibitor and glucocorticoids. The mean serum creatinine and eGFR at end of follow up were significantly lower for the IgAN group (p=0.001 and p=0.02 respectfully) with no difference in the frequency of acute rejection. However, graft and patient survival were not different even after controlling for follow up longer than one decade. During a mean follow up time of 120.8(±49.1) months, IgAN recurrence in the graft was documented in 23(22.5%) patients. The mean time to recurrence was 47.8(41.8) months, with a mean serum creatinine 1.83(±0.52) mg/dl and eGFR of 43.45(±18.4) ml/min/1.73m2 at the end of follow up. Within the IgAN group, although graft function was significantly lower in patients with disease recurrence, compared with that of patients without recurrence (p=0.003), graft loss remained not different between these two subgroups (p=0.49). Conclusion In the newer era of immunosuppression, long-term outcomes of KTx in patients with IgAN, as primary disease, appear significantly improved, and although graft function was significantly lower in patients with IgAN at the end of follow up, graft and patient survival was not different, compared to patients with non-glomerular causes of ESKD. Recurrence of IgAN in the graft was documented in 22.5% of patients and was associated with significantly lower eGFR at end of follow up compared to patients without recurrence but it did not impact graft survival in this time period.