Article
Biochemical Research Methods
Maria A. Willrich, Paula M. Ladwig, Mark A. Martinez, Meera R. Sridharan, Ronald S. Go, David L. Murray
Summary: Ravulizumab is a new therapeutic monoclonal antibody with a longer half-life and potential personalized therapy compared to eculizumab. The study suggests that Ravulizumab has greater affinity for the FcRn immunoglobulin receptor and may have advantages in monitoring complement blockade.
JOURNAL OF IMMUNOLOGICAL METHODS
(2021)
Article
Urology & Nephrology
Sofiane Salhi, David Ribes, Stanislas Faguer
Summary: The article reports a rare case of a patient who developed TMA and acute respiratory distress syndrome after chemotherapy. Although eculizumab successfully treated TMA, the patient eventually progressed to refractory respiratory failure.
CLINICAL KIDNEY JOURNAL
(2021)
Article
Immunology
Ludmila Lupu, Klemens Horst, Johannes Greven, Uemit Mert, Judith A. K. Ludviksen, Kristin Pettersen, Corinna Lau, Yang Li, Annette Palmer, Kang Qin, Xing Zhang, Benjamin Mayer, Martijn van Griensven, Markus Huber-Lang, Frank Hildebrand, Tom Eirik Mollnes
Summary: In this study, the combined inhibition therapy of complement factor C5 and TLR co-receptor CD14 was used to reduce thrombo-inflammation and organ damage in a polytrauma pig model. The therapy significantly decreased the levels of terminal complement complex (TCC), CRP, and IL-6 in the blood. It also prevented platelet consumption and TAT formation, and reduced the requirements for norepinephrine. Moreover, the therapy limited organ damage and reduced the levels of blood lipase, LDH, AST, and NGAL.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Pediatrics
Kazuki Tanaka, Brigitte Adams, Alvaro Madrid Aris, Naoya Fujita, Masayo Ogawa, Stephan Ortiz, Marc Vallee, Larry A. Greenbaum
Summary: The study demonstrated that pediatric patients with aHUS who switched from chronic eculizumab to ravulizumab treatment experienced stable kidney and hematologic parameters, with low occurrence of adverse events.
PEDIATRIC NEPHROLOGY
(2021)
Article
Urology & Nephrology
Gema Ariceta, Bradley P. Dixon, Seong Heon Kim, Gaurav Kapur, Teri Mauch, Stephan Ortiz, Marc Vallee, Andrew E. Denker, Hee Gyung Kang, Larry A. Greenbaum
Summary: Ravulizumab rapidly improved hematologic and kidney parameters in complement inhibitor-naive children with atypical hemolytic uremic syndrome, showing no unexpected safety concerns.
KIDNEY INTERNATIONAL
(2021)
Article
Multidisciplinary Sciences
Ravi Shankar Keshari, Narcis Ioan Popescu, Robert Silasi, Girija Regmi, Cristina Lupu, Joe H. Simmons, Alonso Ricardo, K. Mark Coggeshall, Florea Lupu
Summary: Late-stage anthrax infections involve dysregulated immune responses and hematogenous spread of Bacillus anthracis, leading to severe bacteremia, sepsis, organ failure, and death. Some fulminant anthrax patients develop a secondary atypical hemolytic uremic syndrome (aHUS). By studying baboons challenged with PGN, a pathogen-associated molecular pattern responsible for hemostatic dysregulation in anthrax sepsis, researchers found that C5 neutralization can prevent hemolysis and kidney injury induced by PGN. This study identifies PGN-driven complement activation as the pathologic mechanism underlying hemolytic anemia in anthrax and other gram-positive infections.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
(2021)
Review
Immunology
Nicole Galli, Loredana Pettine, Mauro Panigada, Laura Daprai, Grazia Suriano, Anna Grancini, Wilma Barcellini, Bruno Fattizzo
Summary: This case report highlights the risk of life-threatening infection by non-groupable Neisseria meningitidis in a young patient with paroxysmal nocturnal haemoglobinuria (PNH) treated with ravulizumab. Prompt diagnosis and treatment are crucial in managing such infections in PNH patients on complement inhibitors.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Sarah Julien, Douangsone Vadysirisack, Camil Sayegh, Sharan Ragunathan, Yalan Tang, Emma Briand, Marion Carrette, Laetitia Jean, Rachid Zoubairi, Henri Gonde, Olivier Benveniste, Yves Allenbach, Laurent Drouot, Olivier Boyer
Summary: In a humanized mouse model of IMNM, early administration of zilucoplan was found to prevent the onset of myopathy and significantly inhibit complement activation.
Article
Urology & Nephrology
Thomas Barbour, Marie Scully, Gema Ariceta, Spero Cataland, Katherine Garlo, Nils Heyne, Yosu Luque, Jan Menne, Yoshitaka Miyakawa, Sung-Soo Yoon, David Kavanagh
Summary: Ravulizumab demonstrates long-term efficacy and safety in adults with aHUS, providing additional clinical benefits beyond 6 months of treatment, with most adverse events occurring early during the initial evaluation period and decreasing over time.
KIDNEY INTERNATIONAL REPORTS
(2021)
Article
Immunology
Ahmet Ozen, Nurhan Kasap, Ivan Vujkovic-Cvijin, Richard Apps, Foo Cheung, Elif Karakoc-Aydiner, Bilge Akkelle, Sinan Sari, Engin Tutar, Figen Ozcay, Dilara Kocacik Uygun, Ali Islek, Gamze Akgun, Merve Selcuk, Oya Balci Sezer, Yu Zhang, Gunsel Kutluk, Erdem Topal, Ersin Sayar, Cigdem Celikel, Roderick H. J. Houwen, Aysen Bingol, Ismail Ogulur, Sevgi Bilgic Eltan, Andrew L. Snow, Camille Lake, Giovanna Fantoni, Camille Alba, Brian Sellers, Samuel D. Chauvin, Clifton L. Dalgard, Olivier Harari, Yan G. Ni, Ming-Dauh Wang, Kishor Devalaraja-Narashimha, Poorani Subramanian, Rabia Ergelen, Reha Artan, Sukru Nail Guner, Buket Dalgic, John Tsang, Yasmine Belkaid, Deniz Ertem, Safa Baris, Michael J. Lenardo
Summary: CHAPLE disease is a lethal syndrome caused by genetic loss of the complement regulatory protein CD55. Blocking C5 complement activation with eculizumab can reduce gastrointestinal pathology and restore normal immunity and growth in patients with CHAPLE disease.
Article
Immunology
Emma Ispasanie, Lukas Muri, Marc Schmid, Anna Schubart, Christine Thorburn, Natasa Zamurovic, Thomas Holbro, Michael Kammueller, Gerd Pluschke
Summary: Inhibitors of complement components C5 and/or C3 are used to treat diseases caused by dysregulation of complement activation. However, these inhibitors increase the risk of invasive disease caused by Neisseria meningitidis. In this study, the effect of inhibitors targeting different complement pathways on serum bactericidal activity (SBA) against serogroup B meningococci was compared.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Biology
Wioleta M. Zelek
Summary: The complement system plays a crucial role in innate immunity by recognizing and killing bacteria through opsonization, phagocytosis, and direct lysis. Measuring complement activity in mouse serum is challenging and typically requires a large amount of serum, while utilizing human sera depleted of single complement proteins can be a helpful alternative.
Article
Medicine, General & Internal
Rachel H. Bhak, Nikita Mody-Patel, Scott B. Baver, Colin Kunzweiler, Christopher W. Yee, Sanjana Sundaresan, Natalia Swartz, Mei Sheng Duh, Sangeeta Krishnan, Sujata P. Sarda
Summary: This study compared the effectiveness of C3 complement inhibitor pegcetacoplan and C5 complement inhibitor ravulizumab among PNH patients previously treated with eculizumab. Results showed that patients treated with pegcetacoplan had better outcomes in terms of transfusion avoidance, hemoglobin stabilization, LDH normalization, and blood transfusion reduction compared to patients receiving ravulizumab. Additionally, pegcetacoplan led to improvements in fatigue, global health status, physical functioning, and fatigue symptoms in comparison to ravulizumab.
CURRENT MEDICAL RESEARCH AND OPINION
(2021)
Review
Urology & Nephrology
Zhen Chun Tang, Huang Hui, Chunru Shi, Xiangmei Chen
Summary: This comprehensive review and meta-analysis of clinical trials show that eculizumab can reduce the recurrence of atypical hemolytic uremic syndrome in renal transplant patients, decrease the need for dialysis, improve renal function, and reduce the risk of rejection.
Article
Health Care Sciences & Services
Kelly C. Broderick, James P. Burke, Jesse Fishman, Patrick P. Gleason
Summary: This study aimed to describe real-world treatment patterns and outcomes among PNH patients treated with C5 inhibitors. The study found that some patients continued to experience anemia and hemolysis after treatment, indicating unmet therapy needs. Additionally, the treatment costs for these patients were extremely high.
JOURNAL OF MANAGED CARE & SPECIALTY PHARMACY
(2023)