Article
Immunology
Margarita Lopez-Trascasa, Angel Alonso-Melgar, Marta Melgosa-Hijosa, Laura Espinosa-Roman, Maria Dolores Lledin-Barbancho, Eugenia Garcia-Fernandez, Santiago Rodriguez de Cordoba, Pilar Sanchez-Corral
Summary: Pathogenic gain-of-function mutations in complement Factor B were found to be the cause of atypical HUS in 2007. A four-month-old boy diagnosed with hypocomplementemic aHUS in 2000 suffered severe complications and end-stage renal disease by the age of 3, eventually undergoing a combined liver and kidney transplantation in 2009. After experiencing post-transplant complications, including B-cell non-Hodgkin lymphoma and liver rejection, the patient currently maintains normal kidney and liver functions 12 years post-surgery, suggesting that CLKT was an acceptable therapeutic option.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Immunology
Maryam Saleem, Sana Shaikh, Zheng Hu, Nicola Pozzi, Anuja Java
Summary: Thrombotic microangiopathy (TMA) is a condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ injury caused by endothelial cell damage and microthrombi formation. Membranous nephropathy can be either primary, associated with circulating autoantibodies, or secondary, associated with infections, drugs, cancer, or other autoimmune diseases. Differentiating between primary and secondary TMA as well as primary and secondary membranous nephropathy is crucial for timely treatment, but can be challenging for clinicians, particularly after kidney transplant.
FRONTIERS IN IMMUNOLOGY
(2022)
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)
Review
Medicine, General & Internal
Ana Avila, Eva Gavela, Asuncion Sancho
Summary: Thrombotic microangiopathy is a rare but serious complication affecting kidney transplant recipients, characterized by systemic or localized symptoms. Treatment options include plasma exchange and complement blockade.
FRONTIERS IN MEDICINE
(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
Health Care Sciences & Services
Agnieszka Furmanczyk-Zawiska, Anna Kubiak-Dydo, Ewelina Uzarowska-Gaska, Marta Kotlarek-Lysakowska, Katarzyna Salata, Monika Kolanowska, Michal Swierniak, Pawel Gaj, Beata Leszczynska, Maria Daniel, Krystian Jazdzewski, Magdalena Durlik, Anna Wojcicka
Summary: Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by genetic defects, with variable clinical outcomes, including renal failure. This study presents cases of five family members affected by aHUS, demonstrating the highly variable penetrance of the disease.
JOURNAL OF PERSONALIZED MEDICINE
(2021)
Review
Immunology
Yoko Yoshida, Hiroshi Nishi
Summary: This article introduces the importance of the complement system in innate immunity and its interaction with platelets, neutrophils, endothelial cells, and other components. Complement dysregulation may lead to vascular injury and thrombosis in the kidneys, and further investigation is needed to develop specific therapeutic approaches.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Urology & Nephrology
Yasar Caliskan, Seda Safak, Ozgur Akin Oto, Arzu Velioglu, Berna Yelken, Safak Mirioglu, Ahmet Burak Dirim, Abdulmecit Yildiz, Nurana Guller, Halil Yazici, Alparslan Ersoy, Aydin Turkmen, Krista L. Lentine
Summary: This study compared the clinical outcomes and outcomes of living donation in recipients with aHUS and C3G to a control group. It found that donors for complement-related kidney diseases did not experience major cardiac events or thrombotic microangiopathy, and the occurrence of new-onset hypertension was similar. Although there were two cases of cancer and death among related donors, the overall results suggest that kidney transplantation is relatively safe.
JOURNAL OF NEPHROLOGY
(2023)
Article
Urology & Nephrology
Sjoerd A. M. E. G. Timmermans, Jan G. M. C. Damoiseaux, Alexis Werion, Chris P. Reutelingsperger, Johann Morelle, Pieter van Paassen
Summary: Thrombotic microangiopathy (TMA) syndromes represent severe endothelial damage caused by various mechanisms. The study found that assessment of serum-induced ex vivo C5b9 formation and screening for rare variants in complement genes can better categorize TMA, with complement playing a major role in driving the disease, indicating potential therapeutic and prognostic implications.
KIDNEY INTERNATIONAL REPORTS
(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
Medicine, General & Internal
Zhen Ren, Stephen J. Perkins, Latisha Love-Gregory, John P. Atkinson, Anuja Java
Summary: Genetic testing in a kidney transplant cohort revealed rare variants in complement proteins associated with TMA and C3G, with approximately 50% classified as variants of uncertain significance. Structural and functional analysis of identified variants in complement factor H were conducted, determining both deleterious and normal functional activity. The study highlights the importance of functional analysis of genetic variants in complex clinicopathologic scenarios, providing insights for clinical decision making after kidney transplantation.
FRONTIERS IN MEDICINE
(2021)
Article
Urology & Nephrology
Caroline Duineveld, Romy N. Bouwmeester, Kioa L. Wijnsma, F. J. Bemelman, J. W. van der Heijden, S. P. Berger, L. P. W. J. van den Heuvel, Nicole C. A. J. van de Kar, Jack F. M. Wetzels
Summary: Kidney transplantation in aHUS patients in the Netherlands is performed without eculizumab prophylaxis, and eculizumab therapy is given in case of posttransplant aHUS recurrence. The study found a recurrence rate of 23% without eculizumab prophylaxis, and eculizumab treatment effectively improved or stabilized kidney function in most patients.
KIDNEY INTERNATIONAL REPORTS
(2023)
Article
Urology & Nephrology
Gianluigi Ardissino, Donata Cresseri, Francesca Tel, Antenore Giussani, Stefania Salardi, Martina Sgarbanti, Bice Strumbo, Sara Testa, Valentina Capone, Samantha Griffini, Elena Grovetti, Massimo Cugno, Mirco Belingheri, Chiara Tamburello, Evangeline Millicent Rodrigues, Michela Perrone, Massimo Cardillo, Grazia Corti, Dario Consonni, Lucrezia Furian, Silvana Tedeschi, Piergiorgio Messa, Claudio Beretta
Summary: This study presents experience with KTx in aHUS patients, showing that prophylactic use of Eculizumab can significantly reduce relapse rates. Tailored timing of Eculizumab doses based on classical complement pathway activity for each patient has also shown positive results.
JOURNAL OF NEPHROLOGY
(2021)
Article
Immunology
Viktor Rydberg, Sigridur Sunna Aradottir, Ann-Charlotte Kristoffersson, Naila Svitacheva, Diana Karpman
Summary: This study describes genetic variants in the Swedish and Norwegian populations associated with atypical hemolytic uremic syndrome (aHUS), C3 glomerulonephropathy (C3G), and immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN). Genetic screening of patients with these conditions revealed a total of 68 different genetic variants or deletions in aHUS patients, 29 genetic variants or deletions in C3G patients, and 5 genetic variants in IC-MPGN patients, with 26 novel variants identified. The findings highlight the importance of genetic screening for diagnostics and treatment in these patients.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Medicine, General & Internal
Sjoerd A. M. E. G. Timmermans, Pieter van Paassen
Summary: Thrombotic microangiopathy (TMA) is a rare and life-threatening condition, often affecting the brain and kidneys. Complement dysregulation plays a crucial role in TMA, particularly in secondary atypical HUS patients presenting with coexisting conditions such as hypertensive emergency, pregnancy, and kidney transplantation, shifting the paradigm of the disease.
JOURNAL OF CLINICAL MEDICINE
(2021)
Review
Rheumatology
Maxime Beydon, Christophe Rodriguez, Alexandre Karras, Alexandre Cez, Cedric Rafat, Noemie Jourde-Chiche, Olivier Fain, Carole Philipponnet, Xavier Puechal, Antoine Dossier, Nicolas Dupin, Dan Levy, Ines Aureau, Loic Guillevin, Benjamin Terrier
Summary: Through a case series and literature review, it was found that Bartonella infection can mimic PR3-ANCA-associated vasculitis with involvement of the kidneys and endocarditis, while Coxiella infection may induce vasculitis affecting vessels of all sizes, with 75% presenting as cryoglobulinaemia vasculitis.
Article
Surgery
Hannah Kaminski, Nassim Kamar, Olivier Thaunat, Nicolas Bouvier, Sophie Caillard, Isabelle Garrigue, Dany Anglicheau, Jean-Philippe Rerolle, Yannick Le Meur, Antoine Durrbach, Thomas Bachelet, Helene Savel, Roxane Coueron, Jonathan Visentin, Arnaud Del Bello, Isabelle Pellegrin, Julie Dechanet-Merville, Pierre Merville, Rodolphe Thiebaut, Lionel Couzi
Summary: This study found that everolimus can decrease the incidence of CMV DNAemia and disease in CMV seropositive solid organ transplant recipients.
AMERICAN JOURNAL OF TRANSPLANTATION
(2022)
Article
Urology & Nephrology
Dorian Nezam, Raphael Porcher, Francois Grolleau, Pauline Morel, Dimitri Titeca-Beauport, Stanislas Faguer, Alexandre Karras, Justine Solignac, Noemie Jourde-Chiche, Francois Maurier, Hamza Sakhi, Khalil El Karoui, Rafik Mesbah, Pierre Louis Carron, Vincent Audard, Didier Ducloux, Romain Paule, Jean-Fracois Augusto, Julien Aniort, Aurelien Tiple, Cedric Rafat, Severine Beaudreuil, Xavier Puechal, Pierre Gobert, Ziad Massy, Catherine Hanrotel, Stephane Bally, Nihal Martis, Cecile-Audrey Durel, Geoffroy Desbuissons, Pascal Godmer, Aurelie Hummel, Francois Perrin, Antoine Neel, Claire De Moreuil, Tiphaine Goulenok, Dominique Guerrot, Steven Grange, Aurelie Foucher, Alban Deroux, Carole Cordonnier, Celine Guilbeau-Frugier, Anne Modesto-Segonds, Dominique Nochy, Laurent Daniel, Anissa Moktefi, Marion Rabant, Loic Guillevin, Alexis Regent, Benjamin Terrier
Summary: The study found that PLEX did not improve the primary outcome in the entire population, but identified a subset of patients who could benefit from PLEX. However, these findings need to be validated before being used in clinical decision making.
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
(2022)
Article
Rheumatology
Noemie Jourde-Chiche, Nathalie Costedoat-Chalumeau, Karine Baumstarck, Anderson Loundou, Laurence Bouillet, Stephane Burtey, Valerie Caudwell, Laurent Chiche, Lionel Couzi, Laurent Daniel, Christophe Deligny, Bertrand Dussol, Stanislas Faguer, Pierre Gobert, Guillaume Gondran, Antoine Huart, Aurelie Hummel, Emilie Kalbacher, Adexandre Karras, Marc Lambert, Veronique Le Guern, Ludivine Lebourg, Sandrine Loubiere, Helene Maillard-Lefebvre, Francois Maurier, Micheline Pha, Viviane Queyrel, Philippe Remy, Francoise Sarrot-Reynauld, David Verhelst, Eric Hachulla, Zahir Amoura, Eric Daugas
Summary: For patients with proliferative lupus nephritis, discontinuation of maintenance immunosuppressive therapy (IST) after 2.3 years is not non-inferior to continuation of IST. However, IST discontinuation is associated with a higher risk of severe systemic lupus erythematosus (SLE) flares.
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Urology & Nephrology
Sophie Chauvet, Jill J. Hauer, Florent Petitprez, Marion Rabant, Paula Vieira Martins, Veronique Baudouin, Yahsou Delmas, Noemie Jourde-Chiche, Alexandre Cez, David Ribes, Sylvie Cloarec, Aude Servais, Mohamad Zaidan, Eric Daugas, Michel Delahousse, Alain Wynckel, Amelie Ryckewaert, Anne Laure Sellier-Leclerc, Olivia Boyer, Eric Thervet, Alexandre Karras, Richard J. H. Smith, Veronique Fremeaux-Bacchi
Summary: C3 glomerulopathy (C3G) is a rare complement-mediated disease. The prognosis of this disease is associated with age, kidney function and genetic variants in complement genes. Blood biomarker analysis can predict the kidney outcome of patients.
KIDNEY INTERNATIONAL
(2022)
Article
Urology & Nephrology
Jean Winterbottom, Roslyn J. Simms, Anna Caroli, Emilie Cornec-Le Gall, Nathalie Demoulin, Monica Furlano, Esther Meijer, Olivier Devuyst, Ron T. Gansevoort, Yannick Le-Meur, Norberto Perico, Roser Torra, Albert C. M. Ong
Summary: This study found that ADPKD patients experience significantly decreased quality of life, particularly in terms of overall health, energy, and physical health, accompanied by chronic pain. This research contributes to a better understanding of the impact of ADPKD on patients' quality of life.
CLINICAL KIDNEY JOURNAL
(2022)
Letter
Urology & Nephrology
Justine Solignac, Noemie Jourde-Chiche
CLINICAL KIDNEY JOURNAL
(2023)
Editorial Material
Ophthalmology
Antoine Morel, Amina Chahrazed Debieb, David Mrejen, Lucile Amrouche, Anne Scemla, Estelle Vilain, Christophe Legendre, Dany Anglicheau, Julien Zuber, Antoine Brezin, Nathalie Chavarot
Summary: This case report describes a kidney transplant recipient who developed anterior and intermediate uveitis with CMV infection under belatacept therapy. CMV infection was confirmed using polymerase chain reaction assays. Belatacept therapy was interrupted and medication treatments were initiated, leading to successful control of inflammation.
EUROPEAN JOURNAL OF OPHTHALMOLOGY
(2023)
Article
Urology & Nephrology
Steicy Sobrino, Chrystelle Abdo, Benedicte Neven, Adeline Denis, Nathalie Gouge-Biebuyck, Emmanuel Clave, Soeli Charbonnier, Tifanie Blein, Camille Kergaravat, Marion Alcantara, Patrick Villarese, Romain Berthaud, Laurene Dehoux, Souha Albinni, Esma Karkeni, Chantal Lagresle-Peyrou, Marina Cavazzana, Remi Salomon, Isabelle Andre, Antoine Toubert, Vahid Asnafi, Capucine Picard, Stephane Blanche, Elizabeth Macintyre, Olivia Boyer, Emmanuell Six, Julien Zuber
Summary: Long-term multilineage hematopoietic donor chimerism can occur in patients who receive a transplanted solid organ with lymphoid tissues, such as the intestine or liver. However, there is currently no evidence for kidney-resident hematopoietic stem cells in any mammal species. In this study, we found that human kidney-derived hematopoietic stem cells can reside in the recipient's bone marrow and replace host counterparts, leading to full donor chimerism of both lymphoid and myeloid lineages.
KIDNEY INTERNATIONAL
(2023)
Article
Environmental Sciences
Latame Adoli, Maxime Raffray, Valerie Chatelet, Cecile Vigneau, Thierry Lobbedez, Fei Gao, Florian Bayer, Arnaud Campeon, Elsa Vabret, Laetitia Laude, Jean-Philippe Jais, Eric Daugas, Cecile Couchoud, Sahar Bayat
Summary: This study aims to understand the potential sex disparities and their causes in kidney transplantation access. It will use both quantitative and qualitative research methods to compare the differences in access to kidney transplantation between women and men at different levels, and explore the views of patients and nephrologists on the disease and transplantation.
INTERNATIONAL JOURNAL OF ENVIRONMENTAL RESEARCH AND PUBLIC HEALTH
(2022)
Review
Hematology
Juliette Leon, Marie-Benedicte LeStang, Rebecca Sberro-Soussan, Aude Servais, Dany Anglicheau, Veronique Fremeaux-Bacchi, Julien Zuber
Summary: Overactivation of the complement alternative pathway is the driving factor in the pathogenesis of primary atypical hemolytic uremic syndrome (aHUS). Dysregulation of the complement is frequently seen in patients with aHUS, pregnancy-related hemolytic uremic syndrome (HUS), and severe hypertension-associated HUS. It is uncertain whether self-limited complement activation in other forms of HUS provides mechanistic clues or is a result of endothelial damage. Novel biomarkers are being developed to establish complement-driven pathogenesis. C5 blockade therapy has significantly improved the management of aHUS patients, while its efficacy in secondary forms of HUS needs further investigation through prospective clinical trials. The increased risk of meningococcal infection due to C5 inhibition must be addressed with appropriate prophylaxis. Treatment duration should be based on an individualized benefit/risk assessment.
AMERICAN JOURNAL OF HEMATOLOGY
(2023)
Article
Rheumatology
Robin Arcani, Elisabeth Jouve, Laurent Chiche, Noemie Jourde-Chiche
Summary: We developed a type 2 score derived from the SF-36 to categorize SLE patients and compared immunological and transcriptomic profiles between groups.
CLINICAL RHEUMATOLOGY
(2023)
Article
Peripheral Vascular Disease
Jean-Michel Halimi, Jean-Baptiste de Freminville, Philippe Gatault, Arnaud Bisson, Benedicte Sautenet, Valentin Maisons, Pascal Vigny, Denis Angoulvant, Laurent Fauchier
Summary: This study investigated the characteristics and prognosis of hypertensive encephalopathy (HE) and found that HE had a poor prognosis with high risks of death and other complications. Hypertension and concomitant stroke significantly influenced the prognosis of HE.
Article
Medicine, General & Internal
Ahsan Alam, Emilie Cornec-Le Gall, Ronald D. Perrone
Summary: This article describes autosomal dominant polycystic kidney disease, its signs and symptoms, diagnosis, and treatment options.
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION
(2023)
Article
Rheumatology
Francois Gaillard, Delphine Bachelet, Cecile Couchoud, Cedric Laouenan, Katell Peoc'h, Quentin Simon, Nicolas Charles, Noemie Jourde-Chiche, Eric Daugas
Summary: This study found that lupus activity decreases after the initiation of maintenance dialysis, but non-severe and severe lupus flares still occur. It emphasizes the importance of continued follow-up of lupus patients by specialists after dialysis initiation.