bluuebunny/crossref_metadata_2025_split huggingface dataset:
P0476THE ABSENCE OF REMISSION PREDICTS MORTALITY IN RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS
Authors: Gil Giraldo Leydy Yohana , Muñoz Ramos Patricia , Fernández Perpen Antonio Carlos , Quiroga Borja 
Published: June 2020

Abstract

Background and Aims
Rapidly progressive glomerulonephritis (RPGN) encompasses a group of diseases with a common histology in which the absence of treatment progress to end-stage renal disease. Induction treatment includes the use of immunosuppressants, and in certain cases, plasmapheresis. The final objective of this study was to determine the long-term prognosis of the RPGN.

Method
A retrospective observational study was conducted, including patients diagnosed with RPGN between 2004 and 2019. Baseline epidemiological data and comorbidities were collected, as well as renal function and treatment at the time of diagnosis. During the follow up [median of 42 (5-101 months)], we analyzed the evolution of renal function, mortality and associated factors.

Results
Forty-three patients (65% women) were included, with a mean age of 70 ± 16 years. At the time of diagnosis, mean creatinine was 4.8 ± 2.6 mg/dl, proteinuria 1094 ± 856 mg/day and 37 patients (86%) presented hematuria Thirty-one patients (72%) presented positivity for antibodies against the neutrophil cytoplasm, 7 (16%) for antibodies against the glomerular basement membrane and 5 (12%) for both. Regarding the induction treatment, 41 patients received cyclophosphamide and corticoids and two patients received rituximab. Seventeen (31%) plasmapheresis were performed with a median of 7 (6-7 sessions).
At 6 months, 55% of the patients presented remission (15 patients complete remission and 8 patients partial remission). The median creatinine was 1.9 (1.2-3.1) mg/dl and the proteinuria was 380 (85 -542) mg/day (p&lt;0.0001 compared to the initial data). At that time, 21% (9) of the patients needed dialysis. Associated factors with the absence of remission were diabetes mellitus (p= 0.016), creatinine at diagnosis (p= 0.002) and the need for hemodialysis at admission (p&lt;0.0001). The only independent predictor of remission was initial creatinine (HR 0.5 [0.3-0.9], p= 0.048). During follow up, renal function improved with a median of creatinine at 18 months of 1.6 (1.2 – 2.9) mg/dl and 1.5 (0.8-2.4) mg/dl at the end. Twelve (28%) patients died during follow up. Associated factors with mortality were age (p=0.02), the need for hemodialysis (p=0.015) and the absence of remission at 6 months (p=0.012) (figure 1). An adjusted model using Cox regression demonstrated that the absence of remission was an independent predictor of mortality (HR 0.2 [0.5-0.8], p= 0.032).

Conclusion
Initial renal function and 6-month remission predicts mortality in the RPGN.
P0480LENALIDOMIDE PLUS DEXAMETHASONE FOR PROLIFERATIVE GLOMERULONEPHRITIS WITH MONOCLONAL IG DEPOSITS
Authors: Zhou Houan , Li Manna , Wang Xia , Zeng Caihong , Huang Xianghua , Chen Zhaohong , Cheng Zhen 
Published: June 2020

Abstract

Background and Aims
To evaluate the efficacy and safety of lenalidomide plus dexamethasone (LD) in patients with proliferative glomerulonephritis with monoclonal Ig deposits (PGNMID).

Method
Retrospectively analyzed the clinicopathological data of 6 PGNMID patients who treated with LD protocol from January 2010 to October 2019.

Results
All of the patients received LD treatment for ≥3 months after renal biopsy. 3 patients achieved renal remission. The renal remission rate was 50%. Light microscopy showed membranoproliferative glomerulonephritis and immunofluorescence showed single kappa type IgG3 was deposited in the mesangial region and the vascular loop. Before taking LD scheme, the median urinary protein were 7.76 (1.27,14.57) g/24h, the median serum creatinine 1.34 (0.8,3.27) mg/dL, the median albumin 34.5 (22.4,37.5) g/L. In 5 patients the concentration of serum free kappa and lambda light chain increased, but no patients with abnormal serum free light chain ratio. Complement C3 deteriorated in 2 patients, 2 cases of patients with monoclonal plasma cells, 0.7% and 0.5%, respectively. Immunofixation electrophoresis suggested that 1 patient had positive serum M protein for kappa type IgG3. At the last follow-up, median urine protein was 3.33 (0.33,11.23) g/24h, median serum creatinine was 1.23 (0.91,1.82) mg/dL, and median albumin was 35.9 (24.5,45.6) g/L. The concentration of 4 patients with elevated serum free light chain was lower than that before taking the drug. Two patients with decreased complement C3 increased to normal concentration at the last follow-up. However, the other two patients had a slight decrease in complement C3. The M spike did not turn negative during the follow-up in one patients. Adverse events included anemia, neutropenia, limb numbness, upper respiratory tract infection.

Conclusion
This study reported that lenalidomide plus dexamethasone may be effective in treating proliferative glomerulonephritis with monoclonal Ig deposits for the first time. More attention needs to be paid to the hematological adverse events during lenalidomide treatment.
P0487ROLE OF THERAPEUTIC PLASMAPHERESIS IN ANCA-ASSOCIATED VASCULITIS
Authors: CHALKIA AGLAIA , GIANNOU PANAGIOTA , Damianaki Katerina , KAPOTA ATHANASIA , THOMAS KONSTANTINOS , TSALAPAKI CHRISTINA , VASILOPOULOS DIMITRIOS , PETRAS DIMITRIOS 
Published: June 2020

Abstract

Background and Aims
The role of plasmapheresis in the overall and renal survival of patients with ANCA-associated vasculitis (AAV) has not been fully elucidated. The aim of this study is to address whether plasmapheresis is associated with improvement in renal function and survival at 12 months in patients with severe manifestations of AAV and to record the indications, adverse events and treatment characteristics.

Method
Retrospective, descriptive study of 28 patients with AAV diagnosed over the last five years in a tertiary center. Patients were included if they had received therapeutic plasmapheresis adjunctive to conventional therapy (steroids and immunosuppressants) for the first episode of AAV or in relapse.

Results
12 patients (75% male) were enrolled with average age at diagnosis 78.5±6.14 years. The patients were followed for a median period of 20 months. In 75% of the patients MPO-ANCA was positive, in 17% ANCA negative and in 8% double positive anti-GBM/ANCA. On admission, all patients had abnormal renal function with average serum creatinine 5mg/dl±2.12 and the majority of patients (9/12) were dialysis dependent. Indications for plasmapheresis were: alveolar hemorrhage in 33%, renal impairment in 25% and combination of the two above in 42%. 11 patients received plasmapheresis for the first episode of AAV and 1 patient for relapse. Plasmapheresis was performed using filtration and fresh frozen plasma as replacement fluid. The mean number of plasmapheresis treatment was 8 (range 1 to 19 days) and the average internal time between admission and first plasmapheresis treatment was 3 days. One patient had a severe allergic reaction resulting in early discontinuation and no episodes of severe infection or death were recorded during plasmapheresis. As far as treatment all patients received concomitant immunosuppressive therapy with cyclophosphamide (CYC) and corticosteroids while Rituximab (RTX) was added in 3 patients (3/12). Alveolar hemorrhage was resolved in all patients (100%). After one year, 75% of the patients had renal recovery (cre=5mg/dl±2.12 vs cre=2.6mg/dl±1.6, p=0.06) and 67% of the patients who required hemodialysis at the time of diagnosis, during the first year became independent of dialysis (75% vs 33%, p=0.5). Finally, survival at the end of the first year was 83% with cancer and ischaemic heart disease being the cause of death.

Conclusion
Plasmapheresis is quite often used in daily clinical practice with remarkable results in dialysis independence and survival, without serious complications. The actual role of plasmapheresis in ANCA-vasculitis therapeutic algorithm will be determined shortly with the expected results of a randomized multicenter study.
P0492ICAM-1 AND VEGF AS BIOMARKERS FOR PREDICTION LUPUS NEPHRITIS FLARE
Authors: Chyzh Konstantin , Ryabceva Tatyana , Soroka Nikolay , Viarshynin Pavel 
Published: June 2020

Abstract

Background and Aims
Systemic lupus erythematosus (SLE) is autoimmune systemic disease of the connective tissue and characterized by the development of severe complications associated with damage to vital organs, in particular the kidneys. One of the important issues is an adequate assessment of the current activity and the objective prediction of the lupus nephritis (LN) flare. For this purpose molecular biomarkers are being searched. The work aim is the analyzing role of the intercellular adhesion molecule (ICAM-1) and vascular endothelial growth factor (VEGF) in the serum and urine of patients with SLE as a biomarker for lupus nephritis flare.

Method
The study group consisted of patients with SLE with LN (n = 23). The control group included patients with SLE without renal pathology (n = 13). The concentration of ICAM-1 and VEGF molecules was determined in blood serum and urine by enzyme-linked immunosorbent assay.

Results
The median concentration of ICAM-1in serum in patients with LN is 822.41 (580.50; 1300.95) ng/ml. That is two times higher than this indicator in the control group (378.97 (356.47; 439.52) ng/ml). The concentration of the VEGF molecule in serum in the group of LN is 91.99 (74.18; 132.58) ng/ml. That is three times higher than in the control group (31.13 (22.32; 132.58) ng/ml). An increase in the blood serum concentration of patients with SLE ICAM ≥577.7 ng/ml will indicate the presence of LN with a sensitivity of 82.61% and a specificity of 83.33%. ROC analysis showed the AUC coefficient is 0.927 (p = 0.0001). An increase in the blood serum in VEGF concentration of ≥66.43 ng/ml in patients with SLE will indicate the presence of LN with a sensitivity of 80.00% and a specificity of 83.33%. ROC analysis showed that the AUC coefficient is 0.916 (p = 0.006).

Conclusion
The results allow to conclude that it is possible to use the ICAM and VEGF concentration in serum and urine as early molecular markers of the LN and LN flare in patients with SLE.
P0504FACTORS ASSOCIATED WITH THE LONG-TERM RENAL OUTCOME OF IDIOPATHIC FOCAL SEGMENTAL GLOMERULOSCLEROSIS WITH NEPHROTIC SYNDROME
Authors: TANAKA HIROAKI , Fujii Takayuki , KOSHIZAKA JUNYA , YAMAUCHI NOBUAKI , MORIMOTO MAYU , TERASAKI NORIKO , SUZUKI SATOSHI 
Published: June 2020

Abstract

Background and Aims
In the treatment of idiopathic focal segmental glomerulosclerosis (FSGS) with nephrotic syndrome, the remission of proteinuria is considered to be an important goal. The partial remission of proteinuria improves renal survival, whereas it may progressively reduce the renal function. A study searched for a novel partial remission more accurately reflecting the long-term renal outcome. The goal of proteinuria reduction for improving the renal prognosis remains to be clarified.
We examined factors associated with the long-term renal outcome of idiopathic FSGS.

Method
Of 148 patients with FSGS diagnosed based on kidney biopsy between 1981 and 2018, a retrospective cohort study was conducted involving 33 who had undergone immunosuppressive therapy for nephrotic syndrome, and had been followed-up for ≥1 year, excluding those with secondary FSGS. We examined the renal prognosis, regarding a 50% decrease in the estimated glomerular filtration rate (eGFR) as an outcome. We calculated the rate of decrease in the urinary protein level 4 and 8 months after the start of treatment, and estimated the rate of decrease associated with renal hypofunction using ROC analysis. Based on the results of ROC analysis, Cox’s proportional hazard analysis was performed using factors contributing to renal hypofunction as covariates.

Results
Concerning the background of the 33 patients, the mean follow-up period was 11.4 years, and there were 24 males. The mean age was 49.8 years, and the mean blood pressure was 100.5 mmHg. The mean urinary protein level, albumin (Alb) level, eGFR, and total cholesterol (TCho) level were 7.4 g/day, 2.1 g/dL, 44.3 mL/min/1.73 m2, and 369 mg/dL, respectively. Corticosteroid therapy was selected in 21 patients, whereas it was combined with steroid pulse therapy in 12. The daily dose of prednisolone was 37.3 mg.
On ROC analysis, the rate of decrease in the urinary protein level after 4 months was 83.1% (AUC: 0.74, sensitivity: 0.80, specificity: 0.74), and that after 8 months was 85.7% (AUC: 0.78, sensitivity: 0.90, specificity: 0.65).
Cox’s proportional hazard analysis, in which the data were adjusted with the sex, blood pressure, urinary protein level at the start of treatment, Alb level, eGFR, and treatment methods, showed that the rate of decrease in the urinary protein level after 4 months was significantly correlated with renal hypofunction: after 4 months: hazard ratio, 0.19 (95%CI: 0.04-0.77); p=0.0202; after 8 months: hazard ratio, 0.34 (95%CI: 0.05-1.37); p=0.1359.

Conclusion
In the treatment of idiopathic FSGS with nephrotic syndrome, the rate of decrease in the urinary protein level 4 months after the start of treatment was correlated with the long-term renal outcome.
P0505COST ANALYSIS OF SCREENING FOR IGA NEPHROPATHY USING NOVEL BIOMARKERS
Authors: Ishida Mami , Matsuzaki Keiichi , Ikai Hiroshi , Suzuki Hitoshi , Suzuki Yusuke 
Published: June 2020

Abstract

Background and Aims
IgA nephropathy (IgAN) is the most common primary chronic glomerulonephritis and a major cause of end-stage kidney disease worldwide. Recently, novel biomarkers including aberrantly glycosylated IgA1 and the glycan-specific antibodies can be measured and will predict the IgAN. However, little is known about the cost-effectiveness of this screening method from the societal perspective. This is the cost analysis of additional novel biomarkers to the conventional IgAN screening strategy.

Method
Cost analysis was carried out to compare the two diagnostic strategies with or without biomarkers. We developed an analytical decision model to estimate the lifetime medical expense of each strategy. The decision tree was started at forty years old with first time hematuria, and simulated the clinical strategy after the screening using the novel biomarkers (Group N: N), and conventional strategy (Group C: C). The result of the analysis is presented as the lifetime medical expenses from societal perspective. Discount was not considered.

Results
Lifetime medical expenses per person during forty years were calculated as 31.6 million JPY (≈287,270 USD) for using the novel biomarkers, 33.9 million JPY (≈307,903 USD) for conventional strategy. They were lower by 2.3 million JPY (≈20,633 USD) in Group N. Moreover, by using the novel biomarkers, the expected value of IgAN led to increase by 5.67 percent points (N: 48.44%, C: 42.77%), and the expected value of the dialysis led to decrease by 0.85 percent points (N: 19.91%, C: 19.06%). One-way sensitive analysis showed that the prevalence of IgAN was the most influential variable. In the sensitivity analysis, the rate of tonsillectomy with steroid pulse therapy and achieved clinical remission was higher, the expected lifetime expenses were reduced by 3.3 million JPY (≈30,355 USD) and the expected value of the dialysis led to decrease by 1.76 percent points (N: 11.80%, C: 13.56%).

Conclusion
Screening for IgAN using novel biomarkers is expected to reduce the lifetime medical expenses.
P0506PATTERNS AND OUTCOMES OF GLOMERULAR DISEASES IN ADULTS PRESENTING TO MANSOURA UNIVERSITY HOSPITAL: A SINGLE-CENTER PROSPECTIVE STUDY
Authors: M. M. Abd-Elaziz Hoda , Nassar Mohammed Kamal , Abdallah Ibrahim Dina , Sayed-Ahmed Nagy 
Published: June 2020

Abstract

Background and Aims
Glomerular diseases (GDs) impose a substantial burden on patients and health care systems, and they are considered a major cause of end-stage renal disease (ESRD) worldwide. Morbidity and mortality have shown to be substantially higher in patients with GDs than in the general population. In Egypt, there is yet no available national registry for GDs, and GDs outcome is not adequately studied. Therefore, it is of considerable interest to study patterns and outcomes of GDs in our locality.

Method
Patients with features suggesting GDs presenting to Mansoura nephrology and dialysis unit (MNDU) were recruited in this study between 1 July 2017 and 30 June 2018, and those with biopsy-proven GDs were prospectively followed up for a minimum of 6 months or until either death or reaching ESRD. ESRD was defined as initiation of long-term dialysis or eGFR persistently &lt;15 ml/min per 1.73 m2 during follow up visits. Enrolled patients were subjected to through history taking and clinical examination. Biopsies were independently examined by two pathologists blinded to the clinical data. The study outcomes were to identify the GDs subtype frequencies, and to identify the frequency of those achieving either renal recovery, progression to ESRD or death from all-cause mortality. Logistic regression analysis was carried out to identify the potential predictors for renal recovery.

Results
Sixty-six patients (21 males and 45 females) with biopsy-proven GDs were included and completed the required period of follow up. The median age of the studied patients was 32 years (IQR 23 - 46.45). Fifty percent of the cases had hypertension and only one patient had was diabetic. The main indications for renal biopsy were unexplained kidney function decline (62%) followed by subnephrotic (23%) and nephrotic presentation (15%). Diffuse proliferative GN followed by MPGN and sclerosing GN were the most frequently encountered histopathological patterns. Primary and secondary GDs constituted 30.3% and 69.7% respectively. Lupus nephritis (LN) was the most common cause of secondary GDs and class IV LN was the most frequently encountered class accounting for 16 cases (51.6%) of patients with lupus. By the end of 6-month follow up, renal recovery was achieved in 30 patients (45.5%), 21 patients (31.8%) progressed to ESRD and 7 patients (10.6%) had died. Urban residency and total renal chronicity score were the most significant predictors of renal recovery.

Conclusion
Diffuse proliferative GN is the most common histopathological pattern, and secondary GDs, particularly those related to lupus, are more frequently encountered than primary GDs in our center. Unfortunately, quick complete recovery is not the rule and is possibly predictable by less chronic changes in biopsy.
P0507ANTI-GLOMERULAR BASEMENT MEMBRANE DISEASE. ABOUT 32 CASES
Authors: Amiri Mohamed lotfi , Trabelsi Raja , Ounissi Mondher , Chargui Soumaya , Jerbi Mouna , Gaied Hanene , Hedri Hafedh , Gorsane Imen , Ben abdallah Taieb , Goucha Rim 
Published: June 2020

Abstract

Background and Aims
Anti-Glomerular basement membrane disease « anti-GBM » is a rare autoimmune disease. It most often results in a rapidly progressive glomerulonephritis syndrome associated with intra-alveolar hemorrhage. The diagnostic confirmation is histological, by the demonstration of linear deposits of immunoglobulins (IgG) along the glomerular basement membrane. The aim of our study was to describe its epidemiological, clinical, biological, immunological, histological, and evolutionary characteristics and to identify the different prognostic factors for predicting patient survival and renal survival.

Method
It was a retrospective, descriptive and analytical study including patients over 16 years old with anti-GBM disease proved by kidney biopsy and followed up over a period of 32 years (January 1985 to July 2017), in Internal Medicine Department of Charles Nicolle Hospital of Tunis.

Results
We collected 32 patients with a sex ratio H/F = 1.13. The mean age at diagnosis was 42 years old with extremes of 18 to 81 years old. The most frequent extra-renal manifestations were pulmonary (53%), neurological (12.5%) and ocular (6%) manifestations. Hematuria associated with proteinuria was constantly found.
The latter was nephritic in 31% of patients. All patients had renal insufficiency on admission, oligo-anuric in 60% of cases. The use of extra-renal treatment at admission was necessary in 75% of patients. On the immunological level, the search for anti-MBG antibodies, performed in 20 patients, was positive in 65% of cases. All our patients had undergone a renal biopsy puncture with direct immunofluorescence study.
Diffuse extra-capillary glomerulonephritis was observed in 93% of patients.
Corticotherapy was initiated in 27 cases, associated with plasma exchange in 21 cases and cyclophosphamide in 17 cases. Nine of our patients died in the first year. Renal outcome was marked by partial remission in 2 cases and end-stage renal failure in 19 cases. In univariate analysis, an age greater than 60 years and the occurrence of respiratory distress were predictors of death.
Oligo-anuria and need dialysis on admission were predictive factors for progression to end-stage renal failure.

Conclusion
Anti-GBM disease is a serious illness. Current therapeutic modalities have significantly improved patient survival. These must be intensive and rapid in order to hope for a favorable renal evolution whose prognosis remains reserved.