bluuebunny/crossref_metadata_2025_split huggingface dataset:
P0367STATIN THERAPY AS A PROTECTOR FACTOR OF VENOUS THROMBOEMBOLIC EVENTS IN PATIENTS WITH ANCA-GLOMERULONEPHRITIS ?
Authors: HENRY NICOLAS , Renaud GANSEY , Assia DJEMA , Giorgina PICCOLI , Jean François SUBRA , Jean François AUGUSTO 
Published: June 2020

Abstract

Background and Aims
The incidence of venous thromboembolisms (VTE) is greatly increased in patients with anti-neutrophil cytoplasmic antibody (ANCA)- associated vasculitis (AAV). However, its incidence and risk factors in patients with ANCA-glomerulonephritis (ANCA-GN) has been poorly analyzed. Thus, the aim of the present study was to assess the frequency, the risk factors and outcomes of patients with VTE after ANCA-GN diagnosis.

Method
Patients from the Maine-Anjou AAV registry with a biopsy showing pauci-immune glomerulonephritis between 01/01/2000 and 01/01/2019, were included. VTE events, the site and delay from AAV diagnosis were retrieved from patient’s medical records.

Results
Among the 165 patients, 133 fulfilled the inclusion criteria and were analyzed. After exclusion of arterio-venous fistulae thrombotic events, VTE episodes were diagnosed in 23/133 (17.3%) at a median delay of 3 months from ANCA-GN diagnosis or relapse. As compared to patients that did not developed VTE, patients with VTE had less frequently PR3 ANCAs (p=0.05), tended to be older (p=0.081), had lower serum albumin at AAV diagnosis (p=0.040) and were less frequently on statin therapy (p=0.028). Univariate cox analysis identified age (HR 1.49 for each 10-year increase, p=0.028), serum albumin at diagnosis (HR 2.21 for each 10g/L decrease, p=0.045), lack of statin therapy (HR 5.26, p=0.008) and rituximab treatment (HR 4.10, p=0.026) to be significantly associated with VTE occurrence. However, after adjustment in the multivariate model, only lack of statin therapy (HR 4.80; p=0.039) was significantly associated with VTE, while serum albumin was borderline (HR 2.20 for each 10 g/L decrease, p=0.067). Patients with VTE developed more frequently end-stage renal disease (p=0.040), but AAV relapse rate and mortality were not significantly different as compared to patients without VTE.

Conclusion
Patients with ANCA-GN are at high risk of VTE, especially within the first months following AAV diagnosis. We identified the lack of statin therapy as an independent risk factors of VTE development in our cohort. Thus, our results suggest that statins may have anti-thrombotic properties in ANCA-GN, which opens new therapeutic perspectives.
P0371DENTAL CARIES INDUCED BY CNM-POSITIVE STREPTOCOCCUS MUTANS DERIVED FROM IGA NEPHROPATHY PATIETNS IN RATS INDUCED GLOPERULONEPHRITIS LIKE IGA NEPHROPATHY
Authors: Nagasawa Yasuyuki , Naka Shuhei , Wato Kaoruko , Nomura Ryota , Misaki Taro , Ito Seigo , Kida Aritoshi , Nanami Yoshimasa , Hasuike Yukiko , Takahiro Kuragano , Matsumoto-Nakano Michiyo , Nakano Kazuhiko 
Published: June 2020

Abstract

Background and Aims
IgA nephropathy (IgAN) is one of most common primary glomerulonephritis, whose pathogenesis had remained unclear. We had reported that Cnm-(+)Streptococcus mutans, a kinds of major dental caries bacteria. Cnm-(+)S. mutans had strong collagen binding ability, resulting in high pathogenicity. We reported that the prevalence of Cnm-(+)S. mutans in IgA nephropathy is significantly higher than that in control (Misaki-T et al, Clin Exp Nephrol 2015; 19:844-50), and that Cnm-(+)S. mutans in IgA nephropathy was associated with proteinuria (Misaki-T et al, Sci Rep 2016). We also reported C.rectus and S. mutans increased proteinuria synergistically (Misaki-T et al, Nephron, 2018). In this point, there is no direct evidence that Cnm-(+)S. mutans could induce IgA nephropathy. In this study, we evaluated the renal lesions in rats which had dental caries induced by Cnm-(+)S. mutans derived from IgA nephropathy patient to confirm the pathogenesis of IgA nephropathy like lesions induced by Cnm-(+)S mutans.

Method
Cmn-(+)S. mutans from saliva of IgA nephropathy patients was orally administrated into 4 weeks old male Sprague-Dawley rats. Addition of sugar to water had been kept during study period to develop dental carits. At week 34 after the infection, blood, urine, and kidney samples were evaluated. Kidney samples were stained by Periodic Acid-Schiff (PAS), IgA, C3. Historical evaluation by electron microscope analysis was also performed.

Results
At week 34, plaque score and dental caries score in infectious rats significantly elevated, compared with control rats (P&lt;0.001) (Figure 1). These findings suggested Cmn-(+)S. mutans from IgA nephropathy patients induced dental caries in rats. PAS staining revealed the significant mesangial proliferation. At week 34, the prevalence of IgA deposition in infections rats was higher than that in control rats (52.4% vs 4%), and the prevalence of C3 deposition in infectious rats was also higher than that in control rats (42% vs 11%) (Figure 2). Incidence of hematuria was also significantly higher in dental caries group than control. Electron microscopy analysis revealed high dense deposit in mesangial areas.

Conclusion
Cmn-(+)S. mutans from saliva of IgA nephropathy patients could induce IgA-dominant infection-associated glomerulonephritis, or IgA nephropathy.
P0376EFFECTS OF HYPERURICEMIA ON THE PROGNOSIS OF IGA NEPHROPATHY AND ITS SEX DIFFERENCE
Authors: Oh Tae Ryon , Choi Hong Sang , Kim Chang Seong , Ma Seong Kwon , Kim Soo Wan , Bae Eun Hui 
Published: June 2020

Abstract

Background and Aims
Hyperuricemia is a potential risk factor for immunoglobulin A nephropathy (IgAN) progression, but its sex-specific effects on IgAN progression remain unclear. This study aimed to determine the effect of serum uric acid on IgAN progression and whether its effect varied according to sex.

Method
A total of 4339 patients were retrospectively analyzed at a university medical school. We assessed the effect of serum uric acid on IgAN progression using Kaplan–Meier survival analyses and Cox proportional hazards models. The study’s primary end point was IgAN progression that was defined as a 50% decline in the estimated glomerular filtration rate or the initiation of dialysis.

Results
On average, the serum uric acid levels were higher in the men than in the women. In the fully adjusted Cox proportional hazards model that considered all subjects, the risk of IgAN progression increased by about 25.6% for every 1 mg/dL increase in the baseline uric acid level. The serum uric acid level was an independent risk factor for IgAN progression in both sexes, but its effect was more pronounced in the women (hazard ratio [HR], 1.383; confidence interval [CI],1.263 to 1.514; P&lt;0.001) than in the men (HR, 1.181; CI, 1.097 to 1.272; P&lt;0.001) (Pinteraction &lt;0.001). A sensitivity analysis involving serum uric acid quartiles generated consistent and robust results.

Conclusion
The serum uric acid level was an independent risk factor for IgAN progression, and its effect was more pronounced among the women compared with that among the men.
P0378SERUM URIC ACID IS ASSOCIATED WITH PROGNOSIS OF LUPUS NEPHRITIS IN FEMALE BUT NOT IN MALE
Authors: Oh Tae Ryon , Choi Hong Sang , Kim Chang Seong , Kim Soo Wan , Bae Eun Hui , Ma Seong Kwon 
Published: June 2020

Abstract

Background and Aims
Lupus nephritis (LN) is one of the major complications of systemic lupus erythematosus. Early intervention in lupus nephritis is known to benefit patients' prognosis. Recent studies have found an association between hyperuricemia and lupus nephritis, but the sex-specific role of uric acid in lupus nephritis remains unclear. The purpose of this study is to elucidate the relationship between lupus nephritis and hyperuricemia, and to investigate sex differences.

Method
We retrospectively analyzed 394 patients who diagnosed with LN by renal biopsy. We assessed the effect of serum uric acid on progression of LN using Kaplan–Meier survival analyses and Cox proportional hazards models. The study’s primary end point was LN progression that was defined as the initiation of dialysis.

Results
The male had a higher mean of serum uric acid level than female. In the overall patient analysis, every 1 mg / dL increase in baseline uric acid level increased the risk of LN progression by about 16.9%. The serum uric acid level was an independent risk factor for LN progression in female (hazard ratio [HR], 1.177; confidence interval [CI],1.024 to 1.353; P&lt;0.001) but not in male (HR, 1.0351; CI, 0.661 to 1.622; P = 0.880). A sensitivity analysis involving serum uric acid tertile generated consistent and robust results.

Conclusion
The serum uric acid level was an independent risk factor for LN progression in female but no in male.
P0384PROTEINURIA SELECTIVITY INDEX MAY PREDICT RITUXIMAB RESPONSE IN MCD AND FSGS
Authors: Allinovi Marco , Trivioli Giorgio , Lugli Gianmarco , Caroti Leonardo , Antognoli Giulia , Cirami Calogero 
Published: June 2020

Abstract

Background and Aims
Proteinuria selectivity index (PSI) predicts steroid responsiveness in minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). Rituximab, a monoclonal antibody targeting CD20, has increasingly recognized as a potential therapy of idiopathic podocytopathies, such as MCD and FSGS, although the mechanism of action is still unrecognized and randomized controlled studies are still lacking. Currently, no tools are available to predict responsiveness to RTX. We explored the role of PSI as a potential predictor of RTX responsiveness in MCD and FSGS.

Method
We analysed cases of biopsy-proven MCD and FSGS followed at one single centre, who received RTX therapy. Baseline data, including proteinuria and PSI, were collected. Proteinuria was considered selective with PSI (clearance of urinary IgG/transferrin ratio) &lt;0.20. Complete remission (CR) was resolution of proteinuria and clinical symptoms, while partial remission (PR) was proteinuria decrease (&gt;50%) and substantial improvement of clinical symptoms.

Results
RTX was administered to 14 patients with MCD and 16 patients with FSGS and, of them, PSI was available before treatment for 10 and 14 patients, respectively. Baseline characteristics of the patients are shown in Table 1. Among the 24 cases with available PSI, CR was reported in 12 and PR in 5, while 9 did not respond to RTX. All patients who achieved CR and PR had selective proteinuria at baseline, while all patients with PSI &gt;0.20 did not respond to RTX (Figure 2). Median relapse-free survival was 25.4 months. Among responders, in 10 out of 19 patients (53%) the relapse occurred between 4 and 60 months from initial treatment. No potentially life-threatening adverse events have been observed.

Conclusion
Selective proteinuria before treatment can predict the response to RTX in adult patients with steroid-dependent and refractory forms of MCD and FSGS.
P0387PATHOLOGICAL FEATURES AND CLINICAL COURSE OF LATE-ONSET LUPUS NEPHRITIS
Authors: Shabaka Amir , Sanchez Alamo Beatriz , Gatius Serena , Cases Corona Clara Maria , Valdes Elena , Dominguez Torres Patricia , Diaz Enamorado Yunayka , Fernandez Juarez Gema Maria 
Published: June 2020

Abstract

Background and Aims
It has been described that the early onset of lupus nephritis (LN) at a young age can determine the clinical course and outcome of the disease, being more aggressive with a higher mortality rate. However, no studies have explored the influence of age at onset of the disease in European cohorts of LN patients, neither have they analyzed treatment outcomes depending on age at presentation. Therefore, the aim of our study was to compare the clinical and histological characteristics of patients with late-onset LN compared to those with an onset at a younger age.

Method
We performed a retrospective observational study of biopsy-proven LN diagnosed between 1981 and 2018. Patients were stratified according to their age at presentation of LN, considering an onset with &gt;50 years old as late-onset. Demographic, clinical and serological data at presentation of systemic lupus and LN, during and at the end of follow-up, as well as extrarenal manifestations, histological characteristics of the kidney biopsy, clinical course, received treatment regimens and related adverse effects were registered.

Results
85 LN patients were included in the study, 10 of whom had late-onset LN (11.7%). Late-onset LN patients presented significantly higher serum C3 and C4 levels at the time of kidney biopsy (92.8 ± 22.4 mg/dl vs 62.5 ± 25.7 mg/dl, p=0.002, and 18.6 ± 5.0 vs. 9.3±5.8 mg/dl, p&lt;0.001 respectively), and had lower eGFR measured by CKD-EPI (58.50±19.79 vs 90.41±30.53,p=0.003), a higher frequency of serositis manifestations (53.45% vs 17.46%, p=0.034), predominantly pleuritis (79.37% vs 51.75% p=0.013). Early-onset LN had predominantly Class IV LN (50% vs 10%, p=0.017), whereas in late-onset LN the most frequent type was Class V LN (50% vs 21.3%, p=0.048). Tubular atrophy was more common in late-onset LN (75% vs 34.9%, p=0.035). The group of patients with late-onset LN achieved complete clinical remission more frequently (87.5% vs 57.1%), but there were no differences in relapses between the groups. No differences were found in treatment regimens among the groups. Late-onset LN patients were more seronegative (100% vs 61.3%, p=0.015) at the end of follow-up. Severe infectious complications related to immunosuppression were significantly higher in late-onset LN patients (62.5% vs 22.2%, p=0.027)

Conclusion
Late-onset LN has a milder complement consumption at presentation compared to early-onset, and Class V LN is its most frequent form of presentation. Complete renal remission is more frequently achieved in late-onset LN compared to those with an early-onset, but present more adverse events associated with immunosuppression, particularly infectious complications.
P0389THE PREVALENCE, CLINICAL AND PATHOLOGICAL CHARACTERISTICS OF PRIMARY FOCAL SEGMENTAL GLOMERULOSCLEROSIS IN TURKISH ADULTS: THE DATA FROM TSN-GOLD ( TURKISH SOCIETY OF NEPHROLOGY GLOMERULAR DISEASES) WORKING GROUP
Authors: Kurultak Ilhan , Gungor Ozkan , Ozturk Savas , Dirim Ahmet Burak , Eren Necmi , Yenigun Ezgi , Ahbap Dal Elbis , Dincer Mevlut Tamer , Bora Feyza , Akgur Suat , Sumnu Abdullah , Dursun Belda , Sipahi Savaş , Çetinkaya Hakkı , Sahin İdris , SAHIN Garip , Yılmaz Mürvet , Vatansever Bulent , Aydin Emre , Ulu Memnune Sena , Gundogan Ali , Üstündağ Sedat , Sayarlioglu Hayriye , Kumru Şahin Gizem , Elcioglu Omer Celal , Aydin Zeki , Selcuk Nedim Yılmaz , Onal Ceren , Oruc Meric , Kucuk Mehmet , Aktas Nimet , Derici Ülver , Suleymanlar Gultekin 
Published: June 2020

Abstract

Background and Aims
Focal segmental glomerulosclerosis (FSGS) is one of the primary glomerulopathies which is the most common cause of end-stage renal disease among all primary glomerular diseases. In adults, nephrotic syndrome develops 10-35% due to FSGS. However, its frequency has been increasing in recent years. This study was aimed to present the data and the prevalence of primary FSGS patients in Turkey.

Method
These data were obtained from the National Multicenter (47 centers) Primary Glomerular Diseases registry system, which was entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database between May 2009 and June 2019. The presented data in this study is cross-sectional and includes the recorded data of the patients in the hospitalization period for the kidney biopsy. These demographic, clinic and pathological data of patients were evaluated with using statistically.

Results
Of the 3875 patients enrolled in the Primary Glomerular Diseases database, 850 (21.9%) had FSGS. The mean age of the patients was 47.1 ± 13.7 and 435 (52%) of patients were male. Two hundred and eighty one (33.1%) had hypertension and 93 (11%) had diabetes. Five hundred and four patients (59.3%) were diagnosed as nephrotic syndrome. At the time of diagnosis, the mean systolic blood pressure was 13.8 ± 18.6 mm Hg and the mean diastolic blood pressure was 81.4± 11.4 mm Hg. The laboratory findings at the time of diagnosis on following; serum creatinine 1.2 ± 0.04 mg / dl, albumin 3.3 ± 0.03 g / dl, and 24 hours urinary proteinuria amounts to 4743 ± 181 mg / day. Demographical and clinical data of the patients at the time of diagnosis were presented on Table 1. In pathological examination; the mean number of glomeruli was 16.8 ± 0.3, global sclerotic glomeruli were 3.1 ± 0.1, and segmental sclerotic glomeruli were 2.4 ± 0.1. Summary of Pathological Findings were presented on Table 2

Conclusion
It is very difficult to determine the absolute incidence and prevalence of primary FSGS. Incidence rates have been reported to range from 0.2 to 1.8 / 100,000 per year in the literature. In a United States study, the most common diagnosis in 2501 kidney biopsies was FSGS (35.9%). According to this database, the prevalence of FSGS in primary glomerular diseases in our country is 21.9%.
P0391STUDY OF ANTI COMPLEMENT FACTOR H MEDIATED DISEASE AT A TERTIARY CARE CENTRE
Authors: Lingaraj Umesh 
Published: June 2020

Abstract

Background and Aims
To study the clinical profile and outcome of patients with anti complement factor H mediated disease at our centre.

Method
We conducted a prospective study over 24 months ( August 2016 to July 2018) to assess the clinical profile and outcome of patients with anti complement factor H mediated disease at our tertiary care centre. We had a total of 18 cases during the study period. All patients were followed up to assess their response to therapy.

Results
A total of 28 cases of atypical HUS were seen during the study period of which anti factor H antibody was elevated in 18 (64.2%). All patients presented with hypertension with active urinary sediments and rapidly progressive renal failure. Mean serum creatinine at presentation was 6.8 +/- 1.2 mg/dl and all patients were oliguric at presentation and required haemodialysis. Anti complement factor H antibody was elevated in all patients with a mean of 549 +/- 90 AU/ml. (normal - 0 to 100 AU/ml).Renal biopsy showed thrombotic microangiopathy in 12 patients (66.6%) while features were suggestive of C3 glomerulopathy in 6 patients (33.3%). All patients received induction with plasmapheresis and pulse steroids followed by oral steroids at 1mg / kg with IV cyclophosphamide 500mg/ m 2 monthly for 6 months . This was followed by maintenance immunosuppression with azathioprine +/- oral steroids. 10 patients (55.5%) attained complete remission. Patients with anti complement factor H mediated disease presenting with TMA had better prognosis compared to patients presenting with C3 glomerulopathy. Also patients in the paediatric age group showed good response to treatment

Conclusion
Our study shows that anti complement factor H mediated disease is more common in the paediatric age group with good response to plasmapheresis and immunosuppression in the form of oral steroids and cyclophosphamide. Patients presenting with TMA have better prognosis compared to C3 glomerulopathy
P0398PREVALENCE OF RED COMPLEX OF PERIODONTAL DISEASE BACTERIA IN TONSILS OF IGA NEPHROPATHY PATIENTS
Authors: Nagasawa Yasuyuki , Nomura Ryota , Misaki Taro , Ito Seigo , Naka Shuhei , Wato Kaoruko , Kida Aritoshi , Nanami Yoshimasa , Hasuike Yukiko , Matsumoto-Nakano Michiyo , Nakano Kazuhiko 
Published: June 2020

Abstract

Background and Aims
IgA nephropathy (IgAN) is one of most common primary glomerulonephritis, whose pathogenesis had remained unclear. We had reported that C.rectus and T.denticola, kinds of major periodontal disease bacteria, in tonsils with IgA nephropathy patients were specific to IgAN patients compared with chronic tonsillitis (Nagasawa-Y et al, Plos One, 2014). We also reported C.rectus and S.mutans increased proteinuria synergistically (Misaki-T et al, Nephron, 2018). Red complex of bacteria related with periodontal disease consists of P.gingivalis, T.denticola, and Tannerella forsythia, which has strongest pathogenicity of periodontal disease (Figure 1). But, in this point there were no report of prevalence of red complex of periodontal bacteria in IgA nephropathy. In this study, we evaluated the periodontal disease bacteria including P.gingivalis, T.denticola, and Tannerella forsythia in tonsils of IgAN patients, and the relationship between these periodontal bacteria clinical features in IgAN patients.

Method
Tonsils were obtained from 23 IgAN patients and 63 chronic tonsillitis patients when the tonsillectomy was operated. mRNAs were extracted from tonsils and the prevalences of P.gingivalis, T.denticola, and Tannerella forsythia were evaluated by RT-PCR using bacteria specific primers. All patients gave the written informed consent which was approved by Hyogo College of medicine.

Results
Average age was 33+-14 in IgAN patients, and the age in control patients was 27+-7. The average proteinuria in IgAN patietns was 0.9+-1.1g/gcre, and average hematuria was (2+). The prevalence of T.denticola was very low in both groups (0%, 1.6% respectively). The prevalence of P.gingivalis in IgA patients was significantly higher than that in control patients (33% vs 3.2%, respectively, P&lt;0.0001), and the prevalence of Tannerella forsythia in IgAN patients was also significantly higher than that in control (28% vs 6.4%, respectively, P&lt;0.05) which had not been reported (Figure 2). The types of cilia of P.gingivalis (fim A types) were also evaluated. Obviously untypeable of fim A, which is usually minor type, was dominant in IgAN patients.

Conclusion
Prevalence of red complex of periodontal disease bacteria, especially P.gingivalis, Tannerella forsythia in IgAN patient was higher than that in control. Red complex of periodontal disease bacteria might have some relationship with pathogenesis of IgAN
P0400DECREASED REGULATORY B CELLS AND THEIR ASSOCIATION WITH CLINICAL RESPONSE IN PATIENTS WITH NEW ONSET LUPUS NEPHRITIS
Authors: Gupta Krishan Lal , Girimaji Ivedita , Rakha Aruna , Kaundal Urvashi , Ramachandran Raja , Rathi Manish , Sharma Aman 
Published: June 2020

Abstract

Background and Aims
Studies in lupus nephritis (LN) have shown impairment in both T regulatory (Treg) number and function. However, the data on B regulatory (Breg) is limited in LN. Our objective was to study Breg and Treg populations in newly diagnosed treatment naïve LN and study their change after initiation of immunosuppression.

Method
Study included 25 patients of newly diagnosed LN of Class III (+V), IV (+V), V and 10 healthy controls (HC). Immunophenotyping was performed for peripheral blood mononuclear cell (PBMCs) samples using fluorochrome labelled monoclonal antibodies for Tregs (CD3+CD4+CD25hiCD127loFoxP3+) and Bregs (CD19+CD5+CD1dhi IL-10+). The cells were expressed as percentage of T and B cells. Each lymphocyte population was analysed at baseline, 2 and 6 months after initiation of immunosuppression which was given as per unit protocol.

Results
Bregs were significantly decreased compared to HC at baseline (p=0.002). With immunosuppression, Bregs showed significant increase at 2 and 6 months (p=0.008) and from baseline to 6 months (p=0.005). Bregs in responders significantly increased from 2 to 6 months (p= 0.017) and from baseline to 6 month (p=0.020), while they did not in non-responders. Tregs did not differ significantly from HC and did not show significant increase at 2 and 6 months, in both responders and non-responders.

Conclusion
Breg population in newly diagnosed LN was significantly reduced and increased significantly with immunosuppression. Bregs had an association with renal remission. This underscores the potential role of Bregs in the pathophysiology of LN and their association with clinical response. This could aid in the design of new immunotherapies for treatment of lupus nephritis.