bluuebunny/crossref_metadata_2025_split huggingface dataset:
P0403INNATE DEFECTS OF COMPLEMENT ALTERNATIVE PATHWAY AMONG LUPUS PATIENTS WITH AND WITHOUT THROMBOTIC MICROANGIOPATHY
Authors: Yue Cai , Cui Quexian , Li Xuemei 
Published: June 2020

Abstract

Background and Aims
Thrombotic microangiopathy (TMA) is a rare and severe complication of systemic lupus erythematosus (SLE). The pathogenesis of TMA among lupus patients is not fully understood yet. The aim of the study was to evaluated innate complement alternative pathway (AP) defects among patients with SLE-TMA.

Method
15 consecutive lupus patients with clinically or pathologically diagnosed TMA from Peking Union Medical College Hospital through Jan 2017 to Dec 2019 were enrolled. Lupus patients with TMA secondary to antiphospholipid antibodies, ADAMTS13 inhibitor, overlap syndrome, infection, malignant hypertension and medications were excluded. The exonic regions of 13 genes including CFH, CFI, CFB, C3, CFHR1-5, MCP, THBD, DGKE, PLG were analysed with next generation sequencing. Mutations were interpreted according to ACMG 2015 guideline. Patients with atypical hemolytic uremic syndrome (aHUS) (n=2), lupus nephritis (LN) without TMA (n=8), and malignant hypertension (n=6) were set as control.

Results
Six AP mutations rated as VUS or likely pathogenic were detected among 5/15 (33.3%) patients with SLE-TMA, while three were detected among 3/8 (37.5%) of patients with LN, one among 1/6 (16.7%) patients with malignant hypertension, and three among 2/2 (100%) of patients with aHUS. There was no significant difference regarding mutation rate among the groups.

Conclusion
There is no significant difference regarding AP mutations among lupus patients with and without TMA. Mechanisms other than innate AP abnormality may be involved in the pathogenesis of TMA among lupus patients.
P0410SIGNIFICANT BURDEN OF DISEASE DURING MAINTENANCE TREATMENT OF ANCA-ASSOCIATED VASCULITIS (AAV) PATIENTS IN REAL WORLD PRACTICE IN EUROPE
Authors: Rutherford Peter , Götte Dieter 
Published: June 2020

Abstract

Background and Aims
ANCA associated vasculitis (AAV) is a relapsing remitting long term condition and patients are at risk of organ damage from both active AAV and its therapy in particular high dose and/or prolonged glucocorticoids (GC). The remission maintenance phase of AAV is critical for good long term outcomes including renal preservation as well as preventing AAV relapse. This retrospective study aimed to examine the definition of maintenance, therapy and clinical outcomes in patients managed in routine practice.

Method
1478 AAV patients (France, Germany, Italy, Spain and UK) managed by 493 physicians (61% Nephrologists) who completed induction therapy for organ or life threatening AAV and initiated maintenance therapy between 2014-16 were studied. Data were collected retrospectively at the time maintenance was determined to begin by the physician and then at 6, 12, 18 and 36 months.

Results
49% had granulomatosis with polyangiitis,; mean age 54.2 years with 56% male. 49% had incident AAV and 51% were studied from the time of a relapse requiring remission induction therapy. 70% received cyclophosphamide and GC and 30% received rituximab and GC as induction treatment with 28% receiving plasma exchange. Physicians defined time of the start of maintenance from induction treatment start with mean of 5.7 months on the basis of fixed time point 40%, starting new drug for maintenance 26%, reaching full remission 26% and no specific criteria 8%. At this time of maintenance start 43% were in full remission vs 50% in partial and 7% refractory. Various maintenance regimes were used, 21% received rituximab (88% 6 monthly and 8% 12 monthly, 4% other) at varying planned doses 34% 1g, 40% 500 mg and 23% 375 mg/m2, 4% other regime. Remission rates varied with patients experiencing disease relapse and many patients experienced adverse events (AE) and infections with prolonged GC use being common. Renal function was relatively unchanged and some patients had worsening eGFR, protein excretion or blood pressure. At the most recent clinical review patients had been followed for a mean of 50.7 months – 6% had died, 38% had relapsed at least once, and 11% required renal replacement therapy. 54% had no vasculitis activity, 26% were ANCA positive without active disease and 19% were still experiencing active disease. 32% were still receiving GCs - 22% of them receiving &gt; 5mg/ day.

Conclusion
Maintenance therapy is variably defined but typically at 6 months from start of remission induction therapy. Achieving full remission and preventing relapse are still clinical problems and many patients require ongoing GC therapy to maintain remission. Infectious complications and adverse events are common and renal disease remains an ongoing clinical problem.
P0412VALUE OF INTERNATIONAL RISK-PREDICTION TOOL IN IGA NEPHROAPTHY : A SINGLE CENTRE STUDY
Authors: Arora Dhawal , Kanigicherla Durga , Hamilton Patrick 
Published: June 2020

Abstract

Background and Aims
A new International Risk-Prediction Tool in IgA Nephropathy developed was recently developed to help predict disease progression over a 5-7 year period1. We tested use of this Risk-Prediction Calculator in patients diagnosed with IgA Nephropathy to stratify the risk and predict outcomes during follow up.

Method
All adult patients (aged ≥18years) with biopsy proven IgA Nephropathy diagnosed between 2011 and 2016 at Manchester University NHS Foundation Trust were included in the study. Exclusion criteria included patients with secondary causes, prior exposure to immunosuppression, or presentation eGFR of less that 15ml/min. Demographic, clinical phenotypic & renal histological characteristics (MEST score) at baseline were used to calculate the individual risk scores. These risk scores were compared with outcomes seen during subsequent follow up. Primary outcome was a composite of first ESRD or reduction in eGFR to below 50% of value at biopsy. Patients were censored at last follow visit for primary outcome or death. Renal outcomes were analysed using Kaplan Meier survival plots of subgroups based on predicted risk (&lt;16th percentile, 16-50th percentile, 50-84th percentile or &gt;84th percentile).

Results
121 patients were included in the analyses. 45 other patients were excluded based on exclusion criteria. 84 patients (69%) were males, mean age was 42±16 years, eGFR was 63±34ml/min and uPCR was 151±42mg/mmol. Mean follow up was 51.4±28 months.
87% were on RAS inhibition at or within 6 weeks of kidney biopsy diagnosis. During the follow up period 23 patients (19%) developed the primary outcome. Outcomes were significantly worse in patients with higher risk-prediction scores (Fig 1 log rank p&lt;0.01).

Conclusion
This single centre study confirms that the International IgA Nephropathy Risk-Prediction Model for kidney outcomes can be a valuable tool for prognostication in Primary IgA Nephropathy in routine clinical practice.
P0428CYTOMEGALOVIRUS INFECTION IN PATIENTS WITH GLOMERÜLER DISEASE TREATED WITH CYCLOPHOSPHAMIDE: A SINGLE-CENTER EXPERIENCE
Authors: Kumru Şahin Gizem , Eyüpoğlu Şahin , Eren Sadioğlu Rezzan , Çınar Güle , Ateş Kenan , Ertürk Şehsuvar , Nergizoğlu Gökhan , Şengül Şule , Kutlay Sim , Keven Kenan 
Published: June 2020

Abstract

Background and Aims
Cytomegalovirus (CMV) infection is an important complication in immunocompomised patients. Although approach to CMV infection is well-defined in stem cell and solid organ transplant recipients, less is known about the frequency and risk factors of CMV disease in patients with glomerulonephritis (GN). As few studies have shown that cyclophosphamide (CYC) treatment is a risk factor for CMV infection in GN patients, we aimed to describe the frequency and risk factors of CMV infection in GN patients treated with CYC.

Method
268 patients diagnosed GN between January 2017 and November 2019 in Ankara University İbni Sina Hospital, Nephrology Department. We recruited 43 GN patients who were treated with CYC and screened all patients for viral DNA monthly. CMV infection defined by CMV DNA detected &gt;500 copies/µl. Patients developed CMV and no-CMV infection were compared for age, sex, glomerular pathology, renal function and clinical status before and after treatment.

Results
CMV infection was detected in 10 (23,3%) patients at 2±1 (min:1 max:3) months of CYC treatment (Table-1). 7 patients were treated for CMV infection with parenteral ganciclovir, while the other 3 patients with low CMV DNA level (509, 538 and 540 copies/ml) and no disease symptoms were monitored without antiviral treatment. Patients with CMV infection had higher serum creatinine (4,2±3,2 vs. 1,9±1,8 mg/dl, p=0,006), lower estimated glomerular filtration rate (29±11 vs. 65±8 (ml/min/1.73 m2, p=0.016), lower low-density lipoprotein (144±71 vs. 221±83 mg/dl, p=0,012) at diagnosis compared with no-CMV infection patients (Table-2). Also more patients were diagnosed with rapidly proggressive GN (80,0% vs. 27.3%, p=0,007), and secondary GN was the most common GN diagnosis (80,0% vs. 27.3%, p=0.007) in CMV infection group.

Conclusion
CMV infection is a common complication in GN patients treated with CYC. Routine monitoring and prophylaxis should be considered for the patients who have risk factors for CMV infection.
P0430EFFECT OF CORTICOSTEROIDS THERAPY FOR PATIENTS OF IGA NEPHROPATHY WITH CRESCENTS
Authors: Liang Mengjun , Li Aihua , Jiang Zongpei 
Published: June 2020

Abstract

Background and Aims
Patients with IgA nephropathy (IgAN) presented proteinuria≥1g/d and eGFR≥50ml/min/1.73m2 after supportive treatment had been advised 6-month course of corticosteroids therapy. Update of Oxford classification of IgAN had recommended crescents be added to the MEST score for they were predictive of outcome. Whether we should take some more positive therapy for crescents?

Method
We conducted a single-center, retrospective cohort study enrolling 46 patients from 2017.01 to 2018.06, diagnosed with IgAN by renal biopsy. Eligible patients had proteinuria of 0.5∼3.5g/d, eGFR≥30ml/min/1.73m2 and crescent proportion &lt;50%. Patients were divided into two groups, one for classical steroid treatment (intravenous methylprednisolone 0.25g/d for 3 days at the beginning of months 1, 3 and 5, plus oral prednisone 0.5 mg/kg/d for 6 months, called 1-3-5 Group) and the other assigned an optimized steroid therapy (intravenous methylprednisolone 0.25g/d for 3 days at the beginning of months 1, 2 and 3, plus oral prednisone 0.5 mg/kg/d for 6 months, called 1-2-3 Group). The primary endpoint was remission of proteinuria, secondary endpoint was deterioration in renal function.

Results
There were 23 patients in each group and no significant differences in age, gentle, baseline proteinuria and eGFR between the two groups, except for the proportion of crescents (for Oxford C1 and C2: 52.5% and 13% in 1-3-5 Group vs. 95.7% and 4.3% in 1-2-3 Group respectively, p=0.001). After 6 months therapy, proteinuria in 1-3-5 Group was 0.5(0.2,0.8)g/d (vs. 1.2(0.8,2.6)g/d at baseline, p &lt;0.001) and that in 1-2-3 Group was 0.3(0.2,0.6)g/d (vs. 1.5(0.7,2.6)g/d at baseline, p &lt;0.001). 78.3% of patients in 1-3-5 Group had got remission of proteinuria, while 95.7% in 1-2-3 Group (p=0.187). The 6th month eGFR in 1-3-5 Group was 80.7(59.8,116.2)ml/min/1.73m2 (vs. 77.5(54.8,104.6)ml/min/1.73m2 at baseline, p=0.212), while that in 1-2-3 Group was 97.8(68.6,130.9)ml/min/1.73m2 (vs. 79.5(52.9,108.7)ml/min/1.73m2 at baseline, p=0.002). The slope of eGFR in 1-3-5 Group was 0.7(-1.7,3.3)ml/min/1.73m2/month, while that in 1-2-3 Group was 3(1.2,5.4)ml/min/1.73m2/month, p=0.027. For side effects, two patients in 1-2-3 Group had met bronchitis during the 2nd and 3rh therapy-month respectively; in 1-3-5 Group, one patient had got glaucoma during the 2nd therapy-month and the other had happened steroid-induced diabetes mellitus during the 3rd therapy-month.

Conclusion
Our preliminary results had indicated that optimized steroid therapy had equal effect on reducing proteinuria but more significant advantage to protect against renal function deterioration in IgAN with crescents. 1-2-3 month-steroid pulse therapy had not increase the morbidity of irreversible or severe side effects.
P0453BIOSIMILAR RITUXIMAB AND BRAND RITUXIMAB: RELAPSES AND PROTEINURIA IN THE GLOMERULAR PRYMARY DISEASES
Authors: Bury Roxana , Agraz Irene , Soler Romeo Maria Jose , Garcia Carro Clara , Espinel Eugenia , Seron Micas Daniel , Montoro Bruno , Varon Carlota 
Published: June 2020

Abstract

Background and Aims
Biosimilar drugs need to prove similar effectiveness to the brand name drug, in order to have their authorization from regulatory agencies. Rituximab initially developed as a treatment for non-Hodgkin lymphoma, is used as a therapeutic alternative to several autoimmune diseases, including primary glomerular diseases.
The objective of this study is to observe if there were differences in terms of effects measured in proteinuria and relapse episodes between rituximab brand versus biosimilar in primary glomerular diseases

Method
This is a retrospective descriptive study that included patients receiving rituximab brand or biosimilar for the first time between March 2018 to March 2019, collecting information from the reported medical records with primary glomerulopathy were included. The collected laboratory data included creatinine, proteinuria, leukocyte and lymphocyte count before (0-60 days before) and after (0-60 days after) administration of rituximab.

Results
A total of 19 patients with primary glomerulopathy were included. Six patients (59-years-old (26-74); 50% female) with baseline 6.52±2.00x10^9/L leucocyte count, 2.28±1.10x10^9/L lymphocyte count, 1.63±1.04 mg/dL creatinine and 6.84±3.36g/24h proteinuria, were treated with biosimilar-rituximab. Thirteen patients (58-years-old (25-81); 30% female) with baseline 9.80±4.62x10^9/L leucocyte count, 1.92±1.13 x10^9/L lymphocyte count, 1.61±0.85 mg/dL creatinine and 5.81±4.55 g/24h proteinuria, were treated with brand-rituximab. After the rituximab administration, these values were 6.13±1.94x10^9/L leucocyte count, 1.30±0.59x10^9/L lymphocyte count, 1.16±1.19 mg/dL creatinine, 3.29±0.58g/24h and proteinuria for the biosimilar group; and 8.77±3.78x10^9/L leucocyte count, 1.67±1.13x10^9/L lymphocyte count, 1.56±1.19 mg/dL creatinine and 3.36±2.20g/24h proteinuria for the brand group. After rituximab administration CD19+ lymphocytes become negative in both groups (5/5 for the biosimilar group; 6/6 for the brand group). There were 2 total remissions, 1 partial remission and 3 without response with the biosimilar and 1 total remission, 5 partial remissions and 7 without response with the rituximab brand. Biosimilar was well tolerated in 6/6 patients and Rituximab brand infection did not develop was well tolerated in 11/13 patients and 4/13 patients showed an episode of infection. No statistically significant results were observed for the response to treatment between both groups.

Conclusion
The biosimilar shows a similar profile in terms of proteinuria and remissions against rituximab mark in the reported follow-up period, however our study is limited since it has a small sample, so a larger study is necessary to demonstrate these results with statistical significance
P0458ADVERSE EVENTS AND INFECTIONS, POTENTIALLY RELATED TO GLUCOCORTICOIDS, REMAIN COMMON DURING MAINTENANCE TREATMENT OF ANCA ASSOCIATED VASCULITIS AND ADD TO CLINICAL AND HEALTHCARE RESOURCE USE BURDEN IN EUROPEAN PATIENTS
Authors: Rutherford Peter , Götte Dieter 
Published: June 2020

Abstract

Background and Aims
ANCA associated vasculitis (AAV) treatment aims to quickly control vasculitis activity and then prevent relapse during the maintenance phase of this long term condition. Patients are at risk from disease and treatment related complications with high dose and/or long term glucocorticoid use being a major concern. This retrospective study examined adverse events, infections and healthcare resource use in patients managed in routine practice.

Method
1478 AAV patients (France, Germany, Italy, Spain and UK) managed by 493 physicians (61% Nephrologists) who completed induction therapy for new or relapsing organ or life threatening AAV and initiated maintenance therapy between 2014-16 were studied. Data were collected at the time maintenance was determined to begin by the physician and then at 6, 12, 18 and 36 months. 51% had microscopic polyangiitis, mean age 54.2 years with 56% male. BVAS was not used routinely but 56% had moderate systemic disease and 44% severe systemic disease at the time of start of induction remission treatment.

Results
78% patients had comorbidity (48% hypertension, 20% dylipidaemia, 17% type 2 diabetes mellitus, 14% depression, 13% COPD/asthma and 9% had BMI &gt; 35) at induction treatment start. Maintenance was defined by physicians as starting approximately 6 months into treatment. AEs potentially linked to GCs were common (values are %, bp = hypertension, DM = diabetes mellitus) during the maintenance phase and many patients remained on long term glucocortioids. Infections were also common, in particular upper (URTI) and lower respiratory tract (LRTI) infection and urinary tract infections (UTI). Hospitilisations still occurred during maintenance period, in particular these are due to infections. Maintenance treatment varied and was changed over follow up (in 13-19% patients at each time point) and in a proportion of patients the change in treatment was precipitated by an adverse event or infection (6 months 21%, 12 months 22%, 18 months 23%, 38 months 26%).

Conclusion
Comorbidity is common in AAV patients and needs to be considered carefully when making treatment decisions. Patients receiving maintenance therapy frequently experience potentially GC related AEs and infections are also common. Healthcare resource use continues in the maintenance phase and this is on the background of comorbidity, less then complete remission rates and high use of long term steroids. New approaches are required to reduce clinical and healthcare system burdens during the maintenance treatment phase of AAV.
P0460ASSOCIATION BETWEEN CAUSATIVE MICROORGANISMS OF INFECTIVE ENDOCARDITIS AND CLINICAL SPECTRUM OF RENAL LESIONS
Authors: Pisaruk Alexandra , Povalyaev Nikita , Sorokina Maria , Domonova Elvira , Kotova Elizaveta , Safarova Nargiz , Karaulova Yulia , Balatsky Alexandr , Efremovtseva Marina , Kobalava Zhanna 
Published: June 2020

Abstract

Background and Aims
The relation between microorganisms and renal lesions in IE has bilateral nature: urinary tract infections may be source of systemic invasion that will cause IE, IE causative microorganisms may cause inflammatory changes in glomeruli or be disseminated in renal parenchyma gematogenously with the formation of the abscesses in case of fragmentation or migration of the vegetations. IE-associated GN - is an immune-mediated GN caused by bacterial infection. More commonly GN presented in cases of streptococcal and staphylococcal IE. For renal embolism is typical association with staphylococcal infection, and there are documented cases of renal infarctions in IE caused by S. pneumonia, and C. burnetti. The effect of microorganisms on other clinical manifestations of kidney damage in IE has not been studied.

Method
The study included 209 patients with verified IE. All patients were performed bacteriological and 81(38,8%) parallel one-moment biomolecular (PCR or PCR with follow-up sequenition) tests. In case of death or cardiac surgery, affected valves (n=15) were tested with the same technique. To asses renal function we calculated glomerular filtration rate with CKD-EPI formula. AKI, AKD and CKD were diagnosed in accordance with present guidelines [KDIGO, 2012].

Results
Renal ivolvement into the pathological process trended higher in patients with Gr- (p=0,016) and few causative microorganisms (p=0,039). There are significant differences in incidence of different renal syndromes in patients with MRSA, Gr- and few causative microorganisms - these patients more often had acute nephritic syndrome (respectively p=0,036; p=0,005; p=0,003) (tab. 1). Impairment in the renal function was significantly associated with MRSA (p=0,012), Gr- (p=0,01) and few causative microorganisms (p=0,003). In addition long-lasting renal impairment was observed: patients with MRSA had median time of worsening of the renal function 9 days (IQR 4-14), with Gram-negative microorganisms median time was 9 days (IQR 5-12), with polyflora 10 days (IQR 7-14). But there were no significant differences in median time of renal function worsening in patients with other causative microorganisms. Incidence of AKI and AKD depending on causative organism did not significantly differ.

Conclusion
Kidney lesions in IE significantly associated with Gr- microorganisms, MRSA and with the presence of 2 or more causative microorganisms. Clinical spectrum of renal lesions is diverse: from the changes in the urine sediment to longstanding kidney dysfunction.
P0472RENAL OUTCOMES OF IDIOPATHIC MEMBRANOUS NEPHROPATHY IN ELDERLY
Authors: Breik Nessrine , Jerbi Mouna , Aoudia Raja , Chargui Soumaya , Guaied Hanen , Gorsane Imen , Goucha Rim , Benabdallah Taieb 
Published: June 2020

Abstract

Background and Aims
Idiopathic membranous nephropathy (IMN) is increasingly seen in older patients but little is known about its treatment and outcomes at long term follow up. The objective of this work is to study the renal survival of IMN in elderly as well as prognostic factors.

Method
We conducted a retrospective descriptive study in the nephrology department at Charles Nicolle hospital over a period of 44 years. All older patients (≥65 years) with histologically proven MN were included in this study. Data collected included demographic, clinical and biological parameters in each patient. Data were entered and analyzed using SPSS software. Chi-squared test with a level of significance of 0.05 was used for the qualitative variables.

Results
Twenty-eight patients were collected. The mean age was 67.03 years (65-78 years) with a male predominance (sex ratio: 2.3) and low socio-economic level in 82.5% of cases. Sixteen patients were smokers (57.14%), 5 ethyl patients (17.8%), diabetes was present in 3 patients (10.7%) and hypertension in 11 patients (39.28%). The clinico-biological picture was dominated by high systolic blood pressure in 21 cases (75%), anasarca in 7 cases (25%), proteinuria in all cases and hematuria in 20 cases (71.14%). Biology revealed nephrotic syndrome (NS) in all cases, hypercholesterolemia in 23 cases (82.14%), high serum creatinine in 14 cases (50%) with an average creatinine level of 127,95 µmol/l, anemia in 17 cases (60.7%) and anti-neutrophil cytoplasmic antibodies were positive in one case. MN was confirmed by a kidney biopsy in all cases. Symptomatic treatment was indicated in all patients. Immunosuppressive therapy was started early in 12 patients (40%) because of the severe NS and the deterioration of renal function. Eight patients (26.6%) received corticosteroids alone, three patients received corticosteroid with mycofenolate mofetil and one patient received corticosteroid with ciclosporin. Fourteen patients received only symptomatic treatment. We noted complete remission in 6 patients (21.42%) and end renal stage disease in 5 patients (17.85%). Our study showed that treatment with angiotensin receptors blockers was associated with better renal survival with a statistically significant difference, (p = 0.011). A reduced kidney function at presentation, tubule-interstitial disease and interstitial fibrosis and tubular atrophy were associated with poor survival with a statistically significant result (p = 0.012) (p=0,011) respectively.

Conclusion
IMN is the most common cause of nephrotic syndrome in elderly patients and it has the same risk for progression as in younger individuals. Decrements in glomerular filtration rate, increased risk for comorbidities, vascular disease and change in pharmacokinetics justify special attention to treatment decisions in elderly individuals with IMN.