MO289IS THERE A ROLE OF INTERSTITIAL CHANGES ON THERAPY OUTCOME AMONG PATIENTS WITH FOCAL-SEGMENTAL GLOMERULAR SCLEROSIS AND MINIMAL CHANGE DISEASE?

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Aleksandar Jankovic ◽  
Petar Djuric ◽  
Ana Bulatovic ◽  
Dragana Markovic ◽  
Snezana Pešić ◽  
...  
Keyword(s):  
Complete Remission ◽  
Partial Remission ◽  
Minimal Change Disease ◽  
Minimal Change ◽  
Therapy Outcome ◽  
Glomerular Sclerosis ◽  
First Line Therapy ◽  
Focal Segmental Glomerular Sclerosis ◽  
Significant Difference ◽  
Immunosuppressive Protocol

Abstract Background and Aims It is well-known that interstitial changes in patients with focal-segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) are linked with disease chronicity and progression. The aim of this study was to analyze is there any connection between interstitial changes and outcome after “first line” therapy in patients with FSGS/MCD. Method From 2014 till 2019, biopsy proven diagnosis of FSGS/MCD was established in 40 patients. Interstitial changes were classified in three groups as following: 0-w/o changes; 1-mild changes; 2-severe changes. Patients with nephrotic syndrome (No=29) were treated with prednisolone (1mg/kg) and after six months we have registered therapy outcome as: CR-complete remission; PR-partial remission; EX-death; NO-no effect. Results Among treated patients (age 50.4±15.3 years, 15 men), CR was achieved in 10 patients (34.5%) and 6 out of these 10 (60%) had no interstitial changes. Partial remission was observed in 11 patients (37.9%), in 4 patients (13.8%) therapy did not have any effect, and 4 patients (13.8%) deceased (table 1). All patients in EX and NO group had interstitial changes. There were no significant difference in age, gender, proteinuria, albuminaemia, creatinine and glycaemia levels between groups except for hemoglobin levels that were significantly lower in EX group than in others (∑ 15.144; p=0.002) and urea levels that were significantly higher in EX group (∑ 138.057; p=0.024). Conclusion Patients with FSGS/MCD respond well on standard immunosuppressive protocol, particularly in absence of interstitial changes what may increase the chance for achieving complete remission.

Idiopathic nephrotic syndrome

2018 ◽  
Author(s):  
Patrick Niaudet ◽  
Alain Meyrier
Keyword(s):  
Nephrotic Syndrome ◽  
Light Microscopy ◽  
Minimal Change Disease ◽  
Idiopathic Nephrotic Syndrome ◽  
Minimal Change ◽  
Glomerular Sclerosis ◽  
Focal Segmental Glomerular Sclerosis ◽  
Diffuse Mesangial Proliferation ◽  
Segmental Glomerular Sclerosis ◽  
Visceral Epithelial Cell

Idiopathic nephrotic syndrome is defined by the combination of massive proteinuria, hypoalbuminaemia, hyperlipidaemia, and oedema, and of non-specific histological abnormalities of the glomeruli. Light microscopy may disclose minimal change disease, diffuse mesangial proliferation, or focal segmental glomerular sclerosis (FSGS). The two main causes of idiopathic nephrotic syndrome are characterized histologically. On electron microscopy the glomerular capillaries show a fusion of visceral epithelial cell (podocyte) foot processes and with the exception of some variants no significant deposits of immunoglobulins or complement by immunofluorescence. In a majority of children only minimal changes are seen on light microscopy. These children are referred to as having ‘minimal change disease’. In adults with idiopathic nephrotic syndrome, lesions of FSGS are more frequent.

Rituximab or Cyclophosphamide in the Treatment of Membranous Nephropathy: The RI-CYCLO Randomized Trial

2021 ◽  
Vol 32 (4) ◽  
pp. 972-982 ◽  
Author(s):  
Francesco Scolari ◽  
Elisa Delbarba ◽  
Domenico Santoro ◽  
Loreto Gesualdo ◽  
Antonello Pani ◽  
...  
Keyword(s):  
Complete Remission ◽  
Adverse Events ◽  
Membranous Nephropathy ◽  
Partial Remission ◽  
Pilot Trial ◽  
Clinical Trial Registry ◽  
Serious Adverse Events ◽  
First Line Therapy ◽  
Registration Number ◽  
Favorable Safety

BackgroundA cyclic corticosteroid-cyclophosphamide regimen is the first-line therapy for membranous nephropathy. Compared with this regimen, rituximab therapy might have a more favorable safety profile, but a head-to-head comparison is lacking.MethodsWe randomly assigned 74 adults with membranous nephropathy and proteinuria >3.5 g/d to rituximab (1 g) on days 1 and 15, or a 6-month cyclic regimen with corticosteroids alternated with cyclophosphamide every other month. The primary outcome was complete remission of proteinuria at 12 months. Other outcomes included determination of complete or partial remission at 24 months and occurrence of adverse events.ResultsAt 12 months, six of 37 patients (16%) randomized to rituximab and 12 of 37 patients (32%) randomized to the cyclic regimen experienced complete remission (odds ratio [OR], 0.4; 95% CI, 0.13 to 1.23); 23 of 37 (62%) receiving rituximab and 27 of 37 (73%) receiving the cyclic regimen had complete or partial remission (OR, 0.61; 95% CI, 0.23 to 1.63). At 24 months, the probabilities of complete and of complete or partial remission with rituximab were 0.42 (95% CI, 0.26 to 0.62) and 0.83 (95% CI, 0.65 to 0.95), respectively, and 0.43 (95% CI, 0.28 to 0.61) and 0.82 (95% CI, 0.68 to 0.93), respectively, with the cyclic regimen. Serious adverse events occurred in 19% of patients receiving rituximab and in 14% receiving the cyclic regimen.ConclusionsThis pilot trial found no signal of more benefit or less harm associated with rituximab versus a cyclic corticosteroid-cyclophosphamide regimen in the treatment of membranous nephropathy. A head-to-head, pragmatic comparison of the cyclic regimen versus rituximab may require a global noninferiority trial.Clinical Trial registry name and registration number:Rituximab versus Steroids and Cyclophosphamide in the Treatment of Idiopathic Membranous Nephropathy (RI-CYCLO),NCT03018535

Sirolimus for patients with connective tissue disease-related refractory thrombocytopenia: a single-arm, open-label clinical trial

Rheumatology ◽  
2020 ◽  
Author(s):  
Chanyuan Wu ◽  
Qian Wang ◽  
Dong Xu ◽  
Mengtao Li ◽  
Xiaofeng Zeng
Keyword(s):  
Complete Remission ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Remission Rate ◽  
Complete Remission Rate ◽  
Severe Side Effect ◽  
Open Label ◽  
First Line Therapy ◽  
Primary Endpoints ◽  
Significant Difference

Abstract Objectives Connective tissue disease-related thrombocytopenia (CTD-TP) is a problematic disorder in clinical practice. Because the first-line therapy of glucocorticoid and/or immunosuppressants is not effective for refractory cases, alternative treatment approaches are urgently needed. The present study investigated the efficacy and safety of sirolimus in refractory CTD-TP patients. Methods This single-centre, single-arm, phase II study enrolled 20 refractory CTD-TP patients between September 2017 and September 2018 (registered on ClinicalTrials.gov: NCT03688191). Oral sirolimus administration was dose-adjusted to maintain a therapeutic range of 6–15 ng/ml for 6 months. The primary endpoints were partial and complete remission rates at 6 months. Results Twelve (60%) patients achieved the primary end point with a 50% complete remission rate after 6 months. Among the 14 SLE patients, the overall response rate was 71.4%, with a complete remission rate of 64.3%. None of the primary Sjögren's syndrome cases responded to sirolimus. There was no significant difference in baseline clinical characteristics or lymphocyte subpopulations between responders and non-responders. No severe side effect was detected during the study. Conclusion Sirolimus is an effective and safe treatment option for refractory CTD-TP patients. Trial registration https://clinicaltrials.gov, NCT03688191.

scholarly journals DIABETES REMISSION RATE IN DIFFERENT BMI GRADES FOLLOWING ROUX-EN-Y GASTRIC BYPASS

2018 ◽  
Vol 31 (1) ◽  
Author(s):  
Daniel COELHO ◽  
Eudes Paiva de GODOY ◽  
Igor MARREIROS ◽  
Vinicius Fernando da LUZ ◽  
Antônio Manuel Gouveia de OLIVEIRA ◽  
...  
Keyword(s):  
Gastric Bypass ◽  
Complete Remission ◽  
Partial Remission ◽  
American Diabetes Association ◽  
Remission Rate ◽  
Intervention Group ◽  
Control Group ◽  
Diabetic Patients ◽  
Surgical Morbidity ◽  
Significant Difference

ABSTRACT Background: Type 2 diabetes mellitus has a high long-term remission rate after laparoscopic Roux-en-Y gastric bypass (LRYGB), but few studies have analyzed patients with BMI<35 kg/m2. Aim: To compare glycemic control after LRYGB between BMI 30-35 kg/m2 (intervention group or IG) and >35 kg/m2 patients (control group or CG) and to evaluate weight loss, comorbidities and surgical morbidity. Methods: Sixty-six diabetic patients (30 in IG group and 36 in CG group) were submitted to LRYGB. Data collected annually after surgery were analyzed with generalized estimating equations. Results: Average follow-up was 4.3 years. There was no statistical difference between groups using complete remission American Diabetes Association criteria (OR 2.214, 95%CI 0.800-5.637, p=0.13). There was significant difference between groups using partial remission American Diabetes Association criteria (p=0.002), favouring the CG group (OR 6.392, 95%CI 1.922-21.260). The higher BMI group also had lower HbA1c levels (-0.77%, 95%CI -1.26 to -0.29, p=0.002). There were no significant differences in remission of hypertension, dyslipidemia and surgical morbidity, while weight was better controlled in the IG group. Conclusion: No differences were found in diabetes complete remission, although greater partial remission and the lower levels of glycated hemoglobin in the BMI >35 kg/m2 group suggest a better response among more obese diabetic patients with LRYGB. In addition, both groups had important metabolic modifications at the expense of low morbidity.

scholarly journals Increased Serum Soluble Urokinase-Type Plasminogen Activator Receptor (suPAR) Levels in FSGS: A Meta-Analysis

2019 ◽  
Vol 2019 ◽  
pp. 1-11 ◽  
Author(s):  
Jiwon M. Lee ◽  
Jae Won Yang ◽  
Andreas Kronbichler ◽  
Michael Eisenhut ◽  
Gaeun Kim ◽  
...  
Keyword(s):  
Plasminogen Activator ◽  
Minimal Change Disease ◽  
Meta Analysis ◽  
Minimal Change ◽  
Adult Patients ◽  
Healthy Controls ◽  
Type Plasminogen Activator ◽  
Urokinase Type Plasminogen Activator ◽  
Significant Difference ◽  
Plasminogen Activator Receptor

Introduction. The soluble urokinase-type plasminogen activator receptor (suPAR) has been found to be elevated in primary focal segmental glomerulosclerosis (pFSGS). However, its usefulness as a biomarker for FSGS remains controversial. We conducted a meta-analysis aiming at investigating the significance of suPAR in diagnosing pFSGS. Methods. Electronic databases (PubMed and EMBASE) were searched to identify studies comparing suPAR levels in FSGS patients and controls, from the earliest available date to May 1, 2018. A random-effects model with standardized mean difference (SMD) was used for meta-analyses. Risk of bias was assessed using the Newcastle-Ottawa quality assessment scale. Results. A total of 187 articles were screened, and the final analysis included 13 articles. In comparison to healthy controls, serum suPAR levels were significantly increased in pFSGS patients (SMD, 1.07, 95% confidence interval (CI) 0.65 to 1.48; participants=814; studies=9, I2=85%). Higher suPAR levels were also found in patients with pFSGS compared to those with minimal change disease (SMD 0.53, 95% CI 0.22 to 0.84). Of note, such a difference was not found in pediatric groups (SMD 0.42, 95% CI -0.13 to 0.96) while it was more evidently noted in adult patients (SMD 1.32, 95% CI 0.90 to 1.74). Serum suPAR levels did not differ between pFSGS patients in remission compared to those in active proteinuric state (SMD 0.29, 95% CI -0.30 to 0.88). Comparison with membranous nephropathy and IgA nephropathy showed no significant difference. Conclusions. Our meta-analysis demonstrated that, in comparison to both healthy controls and controls with minimal change disease, suPAR levels were significantly higher in adult patients with pFSGS. suPAR levels did not differ between pFSGS patients during the initial period of diagnosis and those in remission.

scholarly journals Complete Remission of Minimal Change Disease Following an Improvement of Lung Mycobacterium avium Infection

2016 ◽  
Vol 55 (18) ◽  
pp. 2669-2672 ◽  
Author(s):  
Aoi Yamashiro ◽  
Takahiro Uchida ◽  
Seigo Ito ◽  
Naoki Oshima ◽  
Takashi Oda ◽  
...  
Keyword(s):  
Complete Remission ◽  
Mycobacterium Avium ◽  
Minimal Change Disease ◽  
Minimal Change

scholarly journals Usefulness of the cytokines expression of Th1/Th2/Th17 and urinary CD80 excretion in adult-onset minimal change disease

PeerJ ◽  
2020 ◽  
Vol 8 ◽  
pp. e9854
Author(s):  
Ping Chen ◽  
Yan Chen ◽  
Maoqing Jiang ◽  
Yijun Mo ◽  
Huanhuan Ying ◽  
...  
Keyword(s):  
Minimal Change Disease ◽  
Minimal Change ◽  
Healthy Controls ◽  
Adult Onset ◽  
Gm Csf ◽  
Significant Difference ◽  
Cytokine Levels ◽  
Urine Concentrations ◽  
Age Range

Background Minimal change disease (MCD) is a common form of nephrotic syndrome in adults. However, the molecular mechanism underlying the pathogenesis of MCD remains incompletely understood. In this study, we aimed to investigate the role of the cytokines expression of Th1/Th2/Th17 and urinary CD80 excretion in adult-onset MCD patients. Methods The lymphocyte subsets, 34 cytokine levels of Th1/Th2/Th17, serum and urine concentrations of CD80, and expression of CD80 in glomeruli were analyzed in 28 cases (15 males and 13 females; average age: 34.1 years, age range: 18–56 years), including 10 patients with MCD in relapse, nine patients with MCD in remission and nine healthy controls. Results There was no significant difference of CD3+CD4+ cells proportion among patients with MCD in relapse, MCD in remission and healthy controls (P = 0.802). The cytokine levels of GM-CSF and tumor necrosis factor (TNF)-related activation-induced cytokine (TRANCE) in patients with MCD in relapse increased 1.5 times higher than those in remission. An evident increase in the excretion of urinary CD80 was found in patients with relapsed MCD compared with those in remission (598.4 ± 115.8 vs 81.78 ± 7.04 ng/g creatinine, P < 0.001) and healthy controls (598.4 ± 115.8 vs 67.44 ±  8.94  ng/g creatinine, P < 0.001). CD80 expression was observed in podocyte of MCD patient in relapse by immunofluorescence technique. Conclusions The cytokines GM-CSF and TRANCE are increased and the urinary CD80 levels are elevated in adult-onset MCD patients in relapse, indicating a disorder of Th1/Th2/Th17 balance and that the elevated excretion of CD80 may underlie the pathogenesis and development of adult-onset MCD.

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