- |||||||||| Soliris (eculizumab) / AstraZeneca, Ultomiris IV (ravulizumab IV) / AstraZeneca
Journal: Atypical hemolytic uremic syndrome triggered by mRNA vaccination against SARS-CoV-2: Case report. (Pubmed Central) - Oct 25, 2022
The patient is currently in hematological remission with persistent mild renal insufficiency, continuing treatment with eculizumab/ravulizumab. By this case report, we meant to point out the need for careful monitoring of people after vaccination, as it may trigger immune-mediated diseases, especially in those with predisposing factors.
- |||||||||| Soliris (eculizumab) / AstraZeneca, Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
Journal: Emerging Therapies in Antiphospholipid Syndrome. (Pubmed Central) - Oct 24, 2022
Due to this, mechanism-based therapies for APS are not available, and current management following thrombotic events suggests anticoagulation of indeterminate duration, or for obstetric complications, heparin/low molecular weight heparin and aspirin...However, it appears that hydroxychloroquine may enhance the anticoagulant efficacy of vitamin K antagonists in APS patients, and that rituximab may ameliorate some of the "non-criteria" manifestations of APS...A high incidence of complement regulatory gene mutations, and compelling data concerning the efficacy of eculizumab in CAPS, suggests an important role for complement in this disorder. However, additional work is needed to clarify the role of complement in non-catastrophic APS, though emerging data suggests that complement inhibition may be effective in preventing thrombosis in these patients as well.
- |||||||||| Soliris (eculizumab) / AstraZeneca, Ultomiris IV (ravulizumab-cwvz IV) / AstraZeneca, Ultomiris SC QW (ravulizumab-cwvz SC) / AstraZeneca
Enrollment closed, Phase classification, Head-to-Head: Ravulizumab Subcutaneous (SC) Versus Ravulizumab Intravenous (IV) in Adults With Paroxysmal Nocturnal Hemoglobinuria (PNH) Currently Treated With Eculizumab (clinicaltrials.gov) - Oct 20, 2022
P3, N=136, Active, not recruiting,
The diversity of results from the published trials on the efficacy and safety of AHSCT calls for further investigations on determining the ideal AHSCT conditioning and the characteristics of patients. Withheld --> Active, not recruiting | Phase classification: PN/A --> P3
- |||||||||| Soliris (eculizumab) / AstraZeneca
Journal: Impact of Cataract Surgery on Low Luminance Visual Acuity Deficit Measurements. (Pubmed Central) - Oct 18, 2022
To demonstrate the reproducibility of these visual acuity measurements, we used data from drusen-only eyes previously published in the Complement Inhibition with Eculizumab for the Treatment of Nonexudative Age-Related Macular Degeneration (COMPLETE) study...In the current study, significant increases were found in the PL-BCVA and LL-BCVA measurements, with a change of 7.3 letters and 10.2 letters after cataract surgery (P < 0.001 for both), and a statistically significant decrease in LLVAD measurements was found, with a change of -3.0 letters after cataract surgery (P = 0.002). Because of the variable effect of cataracts on LL-BCVA measurements and the significant change in LLVAD measurements after cataract surgery, investigators should be aware that cataract surgery during a trial will have an unpredictable impact on LLVAD measurements, and pseudophakic and phakic patients should be analyzed separately.
- |||||||||| Review, Journal: What's new in neuromyelitis optica spectrum disorder treatment? (Pubmed Central) - Oct 18, 2022
These monoclonal antibodies are directed against the complement system, CD19 B-cells and the IL6 receptor, respectively. All three have been approved for NMOSD in the US and several other countries worldwide and thus provide convincing treatment options.
- |||||||||| Uplizna (inebilizumab) / Horizon Therapeutics, Mitsubishi Tanabe, Soliris (eculizumab) / AstraZeneca, Enspryng (satralizumab) / Roche
Long-term Burden of Attacks in Patients With AQP4+ NMOSD: A Simulation Study Based on Results of a Network Meta-analysis (ePoster Area) - Oct 18, 2022 - Abstract #ECTRIMS2022ECTRIMS_900;
Based on this Markov model, which extrapolated the results of a prior NMA, eculizumab was associated with the longest attack-free time compared with satralizumab or inebilizumab. Patients receiving eculizumab were also projected to experience the highest number of additional QALYs.
- |||||||||| Soliris (eculizumab) / AstraZeneca, Ultomiris IV (ravulizumab IV) / AstraZeneca
Sensitivity analysis using propensity score methods for primary efficacy outcome in the CHAMPION-NMOSD trial (ePoster Area) - Oct 18, 2022 - Abstract #ECTRIMS2022ECTRIMS_889;
P3
Results from the sensitivity analysis were consistent with those from the primary analysis of CHAMPION-NMOSD, indicating that differences in baseline characteristics between the Rav and external Pbo groups did not affect the treatment effect. High E-values suggest that considerable unmeasured confounding would be needed to fully account for the Rav RRR of 98.6%.
- |||||||||| Soliris (eculizumab) / AstraZeneca, Ultomiris IV (ravulizumab IV) / AstraZeneca, Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
Efficacy subgroup analyses from the phase 3 CHAMPION-NMOSD trial in adults with anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (ePoster Area) - Oct 18, 2022 - Abstract #ECTRIMS2022ECTRIMS_883;
P3
Of 58 patients in the Rav arm, at baseline, 30 were receiving monotherapy and 28 were receiving immunosuppressive therapy (IST): steroids (n=12), azathioprine (n=7), mycophenolate mofetil (n=6) or other (n=3)...Significant differences vs placebo were seen in patients who had previously received rituximab (n=20; RRR: 93.7%; p=0.0078) or had not (n=38; RRR 98.1%; p<0.0001)... The robust treatment effect of Rav on RRR was observed across all pre-specified subgroups, including IST use as well as monotherapy, geographic region, age, race and gender.
- |||||||||| Soliris (eculizumab) / AstraZeneca, Ultomiris IV (ravulizumab IV) / AstraZeneca, Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
Efficacy subgroup analyses from the phase 3 CHAMPION-NMOSD trial in adults with anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (ePoster Theatre 1) - Oct 18, 2022 - Abstract #ECTRIMS2022ECTRIMS_866;
P3
Of 58 patients in the Rav arm, at baseline, 30 were receiving monotherapy and 28 were receiving immunosuppressive therapy (IST): steroids (n=12), azathioprine (n=7), mycophenolate mofetil (n=6) or other (n=3)...Significant differences vs placebo were seen in patients who had previously received rituximab (n=20; RRR: 93.7%; p=0.0078) or had not (n=38; RRR 98.1%; p<0.0001)... The robust treatment effect of Rav on RRR was observed across all pre-specified subgroups, including IST use as well as monotherapy, geographic region, age, race and gender.
- |||||||||| Soliris (eculizumab) / AstraZeneca
VASCULAR DISEASES IN PNH () - Oct 16, 2022 - Abstract #EHOC2022EHOC_42;
10 Therapeutic strategies include terminal complement blockade and bone marrow transplantation. Eculizumab, a monoclonal antibody complement inhibitor, is highly effective and the only licensed therapy for PNH.11 The therapeutic anti-C5 antibody eculizumab (Soliris, Alexion) has proven effective in controlling intravascular hemolysis in vivo, leading to remarkable clinical benefit in a majority of PNH patients.12,13 Yet, persistent C3 activation occurring during eculizumab treatment may lead to progressive deposition of C3 fragments on affected erythrocytes and subsequent C3-mediated extravascular hemolysis, possibly limiting the hematologic benefit of anti-C5 treatment.14,15 Thus, upstream inhibition of the complement cascade seems an appropriate strategy to improve the results of current complement-targeted treatment.16,17
- |||||||||| Soliris (eculizumab) / AstraZeneca
Allograft Loss Secondary to Atypical Hemorrhagic Necrosis () - Oct 15, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_4496;
In addition to the supportive care and plasma exchange, complement dysfunction needs to be addressed with specific monoclonal antibodies such as eculizumab. A study by Levi et al administered eculizumab to 12 renal transplant patients with history of aHUS confirming that eculizumab is highly effective in preventing post-transplantation aHUS recurrence.=Additional therapies are in pipeline for future and hence, these cases need to be diagnosed and treated to avoid graft failure.
- |||||||||| Soliris (eculizumab) / AstraZeneca
Complement Mediated Hemolytic Uremic Syndrome After an Egyptian Vacation () - Oct 15, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_4399;
It is likely that the initial hemorrhagic colitis inflammatory milieu overwhelmed and shifted the alternative complement pathway into activated mode, leading to severe HUS, which improved only after eculizumab therapy. Labs
- |||||||||| Soliris (eculizumab) / AstraZeneca
Desires and Needs of Dutch Atypical Hemolytic Uremic Syndrome Patients and Relatives () - Oct 15, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_4395;
Our knowledge in the biochemical and genetic mechanisms of aHUS is continuously increasing, resulting in the development of targeted treatments such as eculizumab...These insights in the actual needs and desires of Dutch aHUS patients and their relatives will help optimize and personalize aHUS health care. In addition, this study can be an example of a more personalized approach in both research and health care of other rare kidney diseases.
- |||||||||| Soliris (eculizumab) / AstraZeneca
A Case of Crescentic C3 Glomerulonephritis in a 24-Year-Old Male: A Rare Entity () - Oct 15, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_4367;
Case Description 24-year-old male with history of hypothyroidism presented to clinic with throat infection and was treated with amoxicillin...He was started on solumedrol and underwent renal biopsy which showed diffuse crescentic glomerulonephritis (GN) on light microscopy...Hence, a differential diagnosis considering postinfectious GN is mandatory. The therapeutic alternatives available at present can be classified as supportive therapy with angiotensin converting enzyme inhibitors, immunosuppression with steroids, mycophenolate, and anticomplement therapy with eculizumab.
- |||||||||| Soliris (eculizumab) / AstraZeneca
Review, Journal: Complement and myasthenia gravis. (Pubmed Central) - Oct 14, 2022
In this review, evidence for the importance of complement, and possible relationships between autoantigen, autoantibodies, complement activation, and the destruction of the membrane are discussed. More recent insights from the results of the complement-inhibiting therapeutic antibody eculizumab are also described, and the mechanisms connecting antibody binding to complement activation are considered from a structural viewpoint.
- |||||||||| Soliris (eculizumab) / AstraZeneca
Postpartum Complement Mediated Thrombotic Microangiopathy: A Case Report (Exhibit Hall, Orange County Convention Center, West Building) - Oct 13, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_3520;
We hypothesize that reduced WT1 expression, leading to podocytopathy could be one of the pathogenic factors contributing to the occurrence of TMA. Based on this experience, and successful treatment with eculizumab, we want to raise awareness to the to the possibility of WT1 mutations triggering TMA in susceptible patients.
- |||||||||| Soliris (eculizumab) / AstraZeneca
Complement-Mediated Hemolytic Uremic Syndrome Presenting With Nephrotic Range Proteinuria (Exhibit Hall, Orange County Convention Center, West Building) - Oct 13, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_3496;
The mechanism of proteinuria in CM-HUS is poorly defined however endothelial injury or podocytopathy could be suspected. Capillary loop with endothelial cell lift off of glomerular basement membrane with fluffy amorphous deposit in subendothelium
- |||||||||| Thymoglobulin (anti-thymocyte globulin (rabbit)) / Sanofi, Soliris (eculizumab) / AstraZeneca
A Case of Mutation Negative Transplant Associated Thrombotic Microangiopathy Successfully Treated With Eculizumab (Exhibit Hall, Orange County Convention Center, West Building) - Oct 13, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_3263;
Everolimus was switched to Belatacept and Eculizumab...MAC complex or C5b-9can be used as a marker to monitor complement inhibition. Given the rarity, there are no guidelines regarding the dose and duration of therapy with Eculizumab; relapses and renal complications often direct the duration (2) and is an area of ongoing research.
- |||||||||| Soliris (eculizumab) / AstraZeneca
Kidney Biopsy Proven Thrombotic Microangiopathy in a Heart Transplant Recipient (Exhibit Hall, Orange County Convention Center, West Building) - Oct 13, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_3260;
Tacrolimus was held and he was initiated on Everolimus...He continues to be on dialysis and cyclosporine with eculizumab without other non-renal findings of TMA...Discussion The early identification and treatment of TMA in OHT is important in preventing further complications associated with it. Although rare as compared to other solid organ transplants, it is essential to maintain TMA as a differential diagnosis for AKI following OHT.
- |||||||||| Tekturna (aliskiren) / PDL
Aliskiren as an Adjunct Treatment for Recurrent C3 Glomerulonephritis (C3GN) in a Transplant Patient (Exhibit Hall, Orange County Convention Center, West Building) - Oct 13, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_3250;
She received induction with anti-thymocyte globulin, and maintenance immunosuppression was tacrolimus, mycophenolate mofetil (MMF) and prednisone...She developed 1.5 g/d proteinuria 3 years post-transplant while on losartan...Comparison of pre- and post-biopsy data showed a statistical reduction (p<0.01) in the mean (SD,standard deviation) size of deposits [2201 nanometer (SD 1102) vs 884 nm (SD 255)] as well as the density of deposits [3.8 per capillary loop (SD 2.5) vs 2.5 per capillary loop (SD 2.8)] Discussion Traditionally management of C3GN has included immunosuppressive agents including steroids, MMF and a possible role of complement pathway blocker, eculizumab...In this first report we describe achievement of complete remission in a patient with recurrent C3GN after kidney transplant with Aliskiren and MMF intensification. These data support further trials for this relatively cheap therapy in this rare disease space.
- |||||||||| Soliris (eculizumab) / AstraZeneca
Successful Treatment With Eculizumab in Patients With Severe ANCA Vasculitis (Exhibit Hall, Orange County Convention Center, West Building) - Oct 13, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_3092;
The eGFR increased significantly in 7/9 patients, and in 4/6 patients, dialysis could be withdrawn. In severe ANCA vasculitis, eculizumab should be considered for improving outcomes.
- |||||||||| Soliris (eculizumab) / AstraZeneca, Ultomiris IV (ravulizumab IV) / AstraZeneca
A Curious Case of Immunoglobulin Deposition in C3-Dominant Membranoproliferative Glomerulopathy (Exhibit Hall, Orange County Convention Center, West Building) - Oct 13, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_3063;
Given its low immunogenicity and slow tissue clearance, prolonged tissue deposition of the antibody is expected, which may generate confusing results. Knowledge of this finding is important because the detection of Ig-positive monoclonal deposits in patients with clinically suspected glomerular disease could lead to misdiagnoses, especially if therapy was discontinued a while prior to the biopsy and the pathologist was not informed of antecedent eculizumab/ Ravulizumab use and if IgG subclass staining is not performed.
- |||||||||| Soliris (eculizumab) / AstraZeneca, Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
A Unique Presentation of Dense Deposit Disease (Exhibit Hall, Orange County Convention Center, West Building) - Oct 13, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_2991;
From 4/2020-4/2021,she was treated with high dose pulse IV steroids, oral steroids, rituximab, mycophenolate mofetil, azathioprine, and cyclophosphamide...She was started on eculizumab with significant improvement over the last six months...This case shows the importance of repeating a renal bx in non-responsive lupus nephritis or GN patients in general as the initial biopsy may not tell the full story. In our case repeat biopsy revealed a new diagnosis leading to different treatment options.
- |||||||||| Soliris (eculizumab) / AstraZeneca
Early Pregnancy-Associated Atypical Hemolytic Uremic Syndrome (aHUS) (Exhibit Hall, Orange County Convention Center, West Building) - Oct 13, 2022 - Abstract #KIDNEYWEEK2022KIDNEY_WEEK_2616;
aHUS was diagnosed and eculizumab (ECU) was prescribed...Thus, TMA in pregnancy is not always PET or HELLP and aHUS should be considered as the diagnosis particularly for presentations occurring prior to 20 weeks gestation. Episodes of severe PET/ HELLP should trigger TMA workup for underlying complement disorder.
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