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68 results on '"Isabelle Ayoub"'

1. Acute Interstitial Nephritis Triggered by a Novel Anti-CD25 Antibody-Drug Conjugate, Camidanlumab Tesirine

Author: Nicholas L. Li, Karen Flores, Jason Prosek, Sergey V. Brodsky, and Isabelle Ayoub
Subjects: Diseases of the genitourinary system. Urology, RC870-923
Published: 2022
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2. Establishing a Case for Anti-complement Therapy in Membranous Nephropathy

Author: Isabelle Ayoub, John P. Shapiro, Huijuan Song, Xiaolan Lily Zhang, Samir Parikh, Salem Almaani, Sethu Madhavan, Sergey V. Brodsky, Anjali Satoskar, Cherri Bott, Lianbo Yu, Michael Merchant, John Klein, Juan M. Mejia-Vilet, Tibor Nadasdy, Dan Birmingham, and Brad H. Rovin
Subjects: complement protein, membranous nephropathy, proteomic analysis, Diseases of the genitourinary system. Urology, RC870-923
Abstract: Introduction: Membranous nephropathy (MN) is a common cause of adult nephrotic syndrome that progresses to end-stage kidney disease in up to 40% of cases. It is an autoimmune disease characterized by glomerular subepithelial deposits containing IgG. In experimental MN, these deposits activate complement and cause kidney damage. The role of complement in human MN is less clearly defined. To address this, the current study focused on the role of complement in 2 independent primary (p) MN cohorts. Methods: Glomeruli were isolated by laser capture microdissection and analyzed by mass spectrometry, focusing on complement proteins, from kidney biopsy specimens from a pMN cohort (n = 11) and from normal controls (n = 5). Immunohistological staining of kidney biopsy specimens for complement proteins was also done. In a second pMN cohort (n = 13), urine levels of Ba, C5a, and C5b-9 (membrane attack complex [MAC]) were measured. Results: Mass spectrometry identified 8 complement pathway components (C1q, C3, C4, C5, C6, C7, C8, and C9) and 5 complement regulators (complement receptor type 1 [CR1], factor H [FH], FH-related protein 2 [FHR2], vitronectin, and clusterin). All complement levels were significantly higher in the MN groups than in the control group, except the level of CR1, which was lower. All pMN biopsy specimens showed negative or trace staining for C1q, positive staining for C3 and C4, and positive staining for at least 1 component of the lectin pathway. Urine Ba, C5a, and MAC were present in pMN, and their levels correlated (rBa,C5a = 0.87, rBa,MAC = 0.89, and rC5a,MAC = 0.97, P = .001 for each correlation). Conclusion: Elevated glomerular levels of C3, C4, and components of MAC (C5b-9) and absent or decreased levels of the complement regulator CR1, along with increased levels of complement activation products in the urine, support the involvement of complement in the pathogenesis of MN. These data raise the possibility that anti-complement therapies may be effective in some forms of MN.
Published: 2021
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3. Spot Urine Protein/Creatinine Ratio Testing at a Large University Medical Center: Evidence for Overuse of This Low-Value Diagnostic Test

Author: Isabelle Ayoub, JoAnna Williams, Dan Birmingham, and Lee Hebert
Subjects: Diseases of the genitourinary system. Urology, RC870-923
Published: 2020
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4. The Use of Glucocorticoids in Lupus Nephritis: New Pathways for an Old Drug

Author: Juan M. Mejía-Vilet and Isabelle Ayoub
Subjects: glucocorticoids, lupus nephritis, systemic lupus erythematosus, prednisone, methylpredisolone, steroids, Medicine (General), R5-920
Abstract: Glucocorticoids therapy has greatly improved the outcome of lupus nephritis patients. Since their discovery, their adverse effects have counterbalanced their beneficial anti-inflammatory effects. Glucocorticoids exert their effects through both genomic and non-genomic pathways. Differential activation of these pathways is clinically relevant in terms of benefit and adverse effects. Ongoing aims in lupus nephritis treatment development focus on a better use of glucocorticoids combined with immunosuppressant drugs and biologics. Newer regimens aim to decrease the peak glucocorticoid dose, allow a rapid glucocorticoid tapering, and intend to control disease activity with a lower cumulative glucocorticoid exposure. In this review we discuss the mechanisms, adverse effects and recent strategies to limit glucocorticoid exposure without compromising treatment efficacy.
Published: 2021
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5. Patient Outcomes in Renal-Limited Antineutrophil Cytoplasmic Antibody Vasculitis With Inactive Histology

Author: Tessa K. Novick, Min Chen, Jennifer Scott, Frank B. Cortazar, Isabelle Ayoub, Mark A. Little, Zdenka Hruskova, Alan D. Salama, Christian Pagnoux, and Duvuru Geetha
Subjects: Diseases of the genitourinary system. Urology, RC870-923
Abstract: Introduction: Little is known about the anticipated disease course for individuals who present with renal-limited antineutrophil cytoplasmic antibody (ANCA)−associated vasculitis but who lack inflammation on a kidney biopsy. The impact of immunosuppression on renal and overall survival is unknown. Methods: Patients were recruited from 2005 to 2016 from 8 centers worldwide (N = 16) for this descriptive study. All had positive ANCA, elevated serum creatinine with active urine sediment, histologic evidence of pauci-immune glomerulonephritis without active lesions, and had no evidence of extrarenal vasculitis. We describe the characteristics of this cohort and the differences in the clinical, histologic, and therapeutic parameters of those who developed primary outcomes of end-stage renal disease (ESRD) and vasculitis relapse. Results: The cohort was 63% Caucasian, and 75% were men, with a median age of 62 years. At entry, the mean ± SD estimated glomerular filtration rate (eGFR) was 24 ± 20 ml/min per 1.73 m2, and 5 patients required dialysis. Twelve patients received immunosuppressive therapy, 25% experienced disease relapse, and 38% developed ESRD. Patients who developed ESRD had lower baseline eGFRs (8 ± 5 ml/min per 1.73 m2 vs. 35 ± 18 ml/min per 1.73 m2; P = 0.001) and more often required dialysis at presentation (83% vs. 0%; P = 0.001). Patients who relapsed were less likely to receive immunosuppression (25% for the relapsed group vs. 92% for the nonrelapsed group; relative risk: 0.27, risk difference: 67%; P = 0.03). Conclusion: Among these patients, lower initial eGFR and dialysis dependence at presentation might increase the risk for ESRD. Immunosuppression did not affect renal outcomes in this sample of patients but was associated with a reduced risk for vasculitis relapse. More information is needed on factors that predict treatment response in this high-risk group. Keywords: ANCA-associated vasculitis, glomerulonephritis, renal limited vasculitis
Published: 2018
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6. Acute glomerulonephritis with large confluent IgA-dominant deposits associated with liver cirrhosis.

Author: Jessica Hemminger, Vidya Arole, Isabelle Ayoub, Sergey V Brodsky, Tibor Nadasdy, and Anjali A Satoskar
Subjects: Medicine, Science
Abstract: Small glomerular IgA deposits have been reported in patients with liver cirrhosis, mainly as an incidental finding in autopsy studies. We recently encountered nine cirrhotic patients who presented with acute proliferative glomerulonephritis with unusually large, exuberant glomerular immune complex deposits, in the absence of systemic lupus erythematosus (SLE) or monoclonal gammopathy-related kidney disease. Deposits were typically IgA dominant/codominant. Our aim was to further elucidate the etiology, diagnostic pitfalls, and clinical outcomes.We present clinical features and kidney biopsy findings of nine cirrhotic patients with an unusual acute immune complex glomerulonephritis. We also identified native kidney biopsies from all patients with liver cirrhosis at our institution over a 13-year period (January 2004 to December 2016) to evaluate presence of glomerular IgA deposits in them (n = 118).Six of nine cirrhotic patients with the large immune deposits had a recent/concurrent acute bacterial infection, prompting a diagnosis of infection-associated glomerulonephritis and treatment with antibiotics. In the remaining three patients, no infection was identified and corticosteroids were initiated. Three of nine patients recovered kidney function (one recovered kidney function after liver transplant); three patients developed chronic kidney disease but remained off dialysis; two patients became dialysis-dependent and one patient developed sepsis and expired shortly after biopsy. Within the total cohort of 118 patients with cirrhosis, 67 others also showed IgA deposits, albeit small; and 42 patients had no IgA deposits.These cases provide support to the theory that liver dysfunction may compromise clearance of circulating immune complexes, enabling deposition in the kidney. At least in a subset of cirrhotic patients, a superimposed bacterial infection may serve as a "second-hit" and lead to acute glomerulonephritis with exuberant immune complex deposits. Therefore, a trial of antibiotics is recommended and caution is advised before immunosuppressive treatment is offered. Unfortunately, most of these patients have advanced liver failure; therefore both diagnosis and management remain a challenge.
Published: 2018
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7. Colon Necrosis Due to Sodium Polystyrene Sulfonate with and without Sorbitol: An Experimental Study in Rats.

Author: Isabelle Ayoub, Man S Oh, Raavi Gupta, Michael McFarlane, Anna Babinska, and Moro O Salifu
Subjects: Medicine, Science
Abstract: Based on a single rat study by Lillemoe et al, the consensus has been formed to implicate sorbitol rather than sodium polystyrene sulfonate (SPS) as the culprit for colon necrosis in humans treated with SPS and sorbitol. We tested the hypothesis that colon necrosis by sorbitol in the experiment was due to the high osmolality and volume of sorbitol rather than its chemical nature.26 rats underwent 5/6 nephrectomy. They were divided into 6 groups and given enema solutions under anesthesia (normal saline, 33% sorbitol, 33% mannitol, SPS in 33% sorbitol, SPS in normal saline, and SPS in distilled water). They were sacrificed after 48 hours of enema administration or earlier if they were very sick. The gross appearance of the colon was visually inspected, and then sliced colon tissues were examined under light microscopy.1 rat from the sorbitol and 1 from the mannitol group had foci of ischemic colonic changes. The rats receiving SPS enema, in sorbitol, normal saline, distilled water, had crystal deposition with colonic necrosis and mucosal erosion. All the rats not given SPS survived until sacrificed at 48 h whereas 11 of 13 rats that received SPS in sorbitol, normal saline or distilled water died or were clearly dying and sacrificed sooner. There was no difference between sorbitol and mannitol when given without SPS.In a surgical uremic rat model, SPS enema given alone or with sorbitol or mannitol seemed to cause colon necrosis and high mortality rate, whereas 33% sorbitol without SPS did not.
Published: 2015
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8. Plasma Nitration of High-Density and Low-Density Lipoproteins in Chronic Kidney Disease Patients Receiving Kidney Transplants

Author: Ahmed Bakillah, Fasika Tedla, Isabelle Ayoub, Devon John, Allen J. Norin, M. Mahmood Hussain, and Clinton Brown
Subjects: Pathology, RB1-214
Abstract: Background. Functional abnormalities of high-density lipoprotein (HDL) could contribute to cardiovascular disease in chronic kidney disease patients. We measured a validated marker of HDL dysfunction, nitrated apolipoprotein A-I, in kidney transplant recipients to test the hypothesis that a functioning kidney transplant reduces serum nitrated apoA-I concentrations. Methods. Concentrations of nitrated apoA-I and apoB were measured using indirect sandwich ELISA assays on sera collected from each transplant subject before transplantation and at 1, 3, and 12 months after transplantation. Patients were excluded if they have history of diabetes, treatment with lipid-lowering medications or HIV protease inhibitors, prednisone dose > 15 mg/day, nephrotic range proteinuria, serum creatinine > 1.5 mg/dL, or active inflammatory disease. Sera from 18 transplanted patients were analyzed. Four subjects were excluded due to insufficient data. Twelve and eight patients had creatinine < 1.5 mg/dL at 3 and 12 months after transplantation, respectively. Results. Nitrated apoA-I was significantly reduced at 12 months after transplantation (p=0.039). The decrease in apoA-I nitration was associated with significant reduction in myeloperoxidase (MPO) activity (p=0.047). In contrast to apoA-I, nitrated apoB was not affected after kidney transplantation. Conclusions. Patients with well-functioning grafts had significant reduction in nitrated apoA-I 12 months after kidney transplantation. Further studies are needed in a large cohort to determine if nitrated apoA-I can be used as a valuable marker for cardiovascular risk stratification in chronic kidney disease.
Published: 2015
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9. Pregnancy History and Kidney Disease Progression Among Women Enrolled in Cure Glomerulonephropathy

Author: Monica L. Reynolds, Andrea L. Oliverio, Jarcy Zee, Elizabeth M. Hendren, Michelle M. O’Shaughnessy, Isabelle Ayoub, Salem Almaani, Tetyana L. Vasylyeva, Katherine E. Twombley, Shikha Wadhwani, Julia M. Steinke, Dana V. Rizk, Meryl Waldman, Margaret E. Helmuth, Carmen Avila-Casado, Nada Alachkar, Carla M. Nester, Vimal K. Derebail, Michelle A. Hladunewich, and Laura H. Mariani
Subjects: Clinical Research, Nephrology
Abstract: INTRODUCTION: Preeclampsia increases the risk for future chronic kidney disease (CKD). Among those diagnosed with CKD, it is unclear whether a prior history of preeclampsia, or other complications in pregnancy, negatively impact kidney disease progression. In this longitudinal analysis, we assessed kidney disease progression among women with glomerular disease with and without a history of a complicated pregnancy. METHODS: Adult women enrolled in the Cure Glomerulonephropathy study (CureGN) were classified based on a history of a complicated pregnancy (defined by presence of worsening kidney function, proteinuria, or blood pressure; or a diagnosis of preeclampsia, eclampsia, or hemolysis, elevated liver enzymes, and low platelets [HELLP] syndrome), pregnancy without these complications, or no pregnancy history at CureGN enrollment. Linear mixed models were used to assess estimated glomerular filtration rate (eGFR) trajectories and urine protein-to-creatinine ratios (UPCRs) from enrollment. RESULTS: Over a median follow-up period of 36 months, the adjusted decline in eGFR was greater in women with a history of a complicated pregnancy compared to those with uncomplicated or no pregnancies (−1.96 [−2.67, −1.26] vs. −0.80 [−1.19, −0.42] and −0.64 [−1.17, −0.11] ml/min per 1.73 m(2) per year, P = 0.007). Proteinuria did not differ significantly over time. Among those with a complicated pregnancy history, eGFR slope did not differ by timing of first complicated pregnancy relative to glomerular disease diagnosis. CONCLUSIONS: A history of complicated pregnancy was associated with greater eGFR decline in the years following glomerulonephropathy (GN) diagnosis. A detailed obstetric history may inform counseling regarding disease progression in women with glomerular disease. Continued research is necessary to better understand pathophysiologic mechanisms by which complicated pregnancies contribute to glomerular disease progression.
Published: 2023

10. The Diagnostic Conundrum of Glomerular Crescents With IgA Deposits

Author: Mineaki Kitamura, Salem Almaani, Bindu Challa, Mohankumar Doraiswamy, Isabelle Ayoub, Laura Biederman, Samir V. Parikh, Ana Molovic-Kokovic, Jason Benedict, Nilesh Mhaskar, Zeid J. Khitan, Sergey V. Brodsky, Tibor Nadasdy, and Anjali A. Satoskar
Subjects: Nephrology
Published: 2023

11. Membranoproliferative Glomerulonephritis With Changing Immunofluorescence Pattern

Author: Anjali A. Satoskar, Dalia Y. Ibrahim, Sergey V. Brodsky, Isabelle Ayoub, Tibor Nadasdy, and Brad H. Rovin
Subjects: Nephrology
Published: 2022

12. Prediction models of treatment response in lupus nephritis

Author: Jim C. Oates, Betty P. Tsao, Aastha Khatiwada, Brad H. Rovin, Bethany J. Wolf, Linyu Geng, Huijuan Song, and Isabelle Ayoub
Subjects: Oncology, medicine.medical_specialty, Treatment response, Proteinuria, Receiver operating characteristic, business.industry, Lupus nephritis, Renal function, Symptom Flare Up, medicine.disease, Lupus Nephritis, Article, ROC Curve, Urine biomarkers, Nephrology, Internal medicine, Cohort, medicine, Humans, Lupus Erythematosus, Systemic, medicine.symptom, business, Biomarkers, Predictive modelling
Abstract: In order to develop prediction models of one-year treatment response in lupus nephritis, an approach using machine learning to combine traditional clinical data and novel urine biomarkers was undertaken. Contemporary lupus nephritis biomarkers were identified through an unbiased PubMed search. Thirteen novel urine proteins contributed to the top 50% of ranked biomarkers and were selected for measurement at the time of lupus nephritis flare. These novel markers along with traditional clinical data were incorporated into a variety of machine learning algorithms to develop prediction models of one-year proteinuria and estimated glomerular filtration rate (eGFR). Models were trained on 246 individuals from four different sub-cohorts and validated on an independent cohort of 30 patients with lupus nephritis. Seven models were considered for each outcome. Three quarters of these models demonstrated good predictive value with areas under the receiver operating characteristic curve over 0.7. Overall, prediction performance was the best for models of eGFR response to treatment. Furthermore, the best performing models contained both traditional clinical data and novel urine biomarkers, including cytokines, chemokines, and markers of kidney damage. Thus, our study provides further evidence that a machine learning approach can predict lupus nephritis outcomes at one year using a set of traditional and novel biomarkers. However, further validation of the utility of machine learning as a clinical decision aid to improve outcomes will be necessary before it can be routinely used in clinical practice to guide therapy.
Published: 2022

13. Systemic Lupus Erythematosus

Author: Michelle Petri, Martin Aringer, Isabelle Ayoub, Salem Almaani, Hermine Brunner, Maria Dall’Era, Mengdi Jiang, Richard Furie, Jessica Greco, Fiona Goldblatt, Jennifer Huggins, T. W. J. Huizinga, David Isenberg, Nicholas L. Li, R. C. Monahan, Samir V. Parikh, David Pisetsky, Abin P. Puravath, Brad Rovin, Daniel Wallace, Xuan Zhang, and Lidan Zhao
Published: 2023

14. Sparsentan in patients with IgA nephropathy: a prespecified interim analysis from a randomised, double-blind, active-controlled clinical trial

Author: Hiddo J L Heerspink, Jai Radhakrishnan, Charles E Alpers, Jonathan Barratt, Stewart Bieler, Ulysses Diva, Jula Inrig, Radko Komers, Alex Mercer, Irene L Noronha, Michelle N Rheault, William Rote, Brad Rovin, Howard Trachtman, Hernán Trimarchi, Muh Geot Wong, Vlado Perkovic, Eric Alarmartine, Dong Wan Chae, Lucia Del Vecchio, Jurgen Floege, Shang-Jyh Hwang, Bojan Jelakovic, Bart Maes, Robert Malecki, Marius Miglinas, Fernando Eduardo Barbosa Nolasco, Manual Praga, Kannaiyan Rabindranath, Mai Rosenberg, Sydney Chi Wai Tang, Vladmir Tesar, Bhadran Bose, Muralikrishna Gangadharan, Stephen McDonald, Chen Peh, Sadia Jahan, Chii Yeap, Philip Clayton, Georgina Irish, Nikhil Thyagarajan, Peter Hollett, Rathika Krishnasamy, Robert Carroll, Shilpanjali Jesudason, Susan Crail, Toby Coates, Jane Waugh, Euan Noble, Kumaradevan Mahadevan, Victoria Campbell, Tania Salehi, Wai Lim, Neil Boudville, Aron Chakera, Doris Chan, Anoushka Krishnan, Yusuf Eqbal, Alastair Gillies, Eswari Vilayur, Thida Maung Maung Myint, Nicholas Gray, Melissa Cheetham, Carol Pollock, Bruce Cooper, Amanda Mather, Sarah Roxburgh, Yvonne Shen, Stefanie Stangenberg, Amanda Siriwardana, Emma O'Lone, Susan Wan, Brendon Neuen, Jeffrey Tsun Kit Ha, Dana Kim, Lauren Heath, Arunima Jain, Elaine Phua, Yan Li, Martin Gallagher, Meg Jardine, Angus Ritchie, Mona Razavian, Celine Foote, Roger Wyndham, Shaundeep Sen, Zoltan Endre, Jonathan Erlich, Mangalee Fernando, Kenneth Yong, Grant Luxton, Sradha Kotwal, Simon Roger, Vidu Wijeratne, David Packham, Ian Fraser, Bert Vandewiele, Margo Laute, Wim Lemahieu, Sofie Jamar, Sara Ombelet, Gert Meeus, Marc Decupere, Olivier Schockaert, Peter Doubel, Liesbeth Viaene, Luc Radermacher, Catherine Masset, Martial Moonen, Eric Firre, Martina Milicevic, Xavier Warling, An Vanacker, Thomas Malfait, Ivan Durlen, Ivica Horvatic, Ana Savuk, Lana Gellineo, Sandra Karanovic, Zivka Dika, Djuro Plavljanic, Ivana Mikacic, Dubravka Trajbar Kentric, Dunja Barisic, Marija Stankovic, Karolina Majstorovic Barac, Ivan Kruljac, Drasko Pavlovic, Martin Drinkovic, Ingrid Prkacin, Jerko Barbic, Zvonimir Sitas, Dunja Vujcic, Ivan Rychlik, Anna Benesova, Klara Drinovska, Karolina Kratka, Dita Maixnerova, Madis Ilmoja, Kristin Unt, Kadri Lilienthal, Asta Auerbach, Liisi Leis, Julia Piel, Annika Adoberg, Kulli Kolvald, Kristi Veermae, Kadri Telling, Elviira Seppet, Jana Uhlinova, Philippe Zaoui, Pierre-Louis Carron, Ingrid Masson, Miriana Dinic, Damien Thibaudin, Christian Broyet, Nicolas Maillard, Hesham Mohey, Christophe Mariat, Guillaume Claisse, Eric Alamartine, Bertrand Dussol, Stephane Burtey, Noemie Chiche-Jourde, Jean-Emmanuel Serre, Guillaume Jeantet, Leila Chenine, Anne Blanchard, Stephane Roueff, Eric Thervet, David Fouassier, Alexandre Buffet, Marine Livrozet, Roxane Gaisset, Alexandre Karras, Anne-Elisabeth Heng, Cyril Garrouste, Carole Philipponnet, Clementine Nicolo, Alba Atenza, Camille Lanaret, Clarisse Greze, Valentin Mayet, Clement Dumond, Yahsou Delmas, Christian Combe, Claire Rigothier, Laure Burguet, Aurore Labat, Simon Mucha, Valérie de Précigout, Thomas Weinreich, Helmut Reichel, Diliana Draganova, Lothar Wolf, Bernd Hohenstein, Sven Heinrichs, Simone Kulka, Sebahat Sat, Lea Weiland, Thilo Krueger, Gunter Wolf, Christiane Kettner, Mandy Schlosser, Johann Konstantin Herfurth, Annegret Koch, Martin Busch, Stephan Christian Werth, Martin Nitschke, Figen Cakiroglu, Franziska Sarnow, Lisa Schulz, Stefan Weiner, Nikolaus Wirtz, Eric Koester, Marcus Moeller, Eleni Stamellou, Silja Sanden, Hans Schmidt-Guertler, Wanja Bernhardt, Margret Patecki, Georg Schlieper, Kevin Schulte, Annette Girardet, Ulrich Kunzendorf, Lorraine Pui Yuen Kwan, Maggie Ming Yee Mok, Gary Chi Wang Chan, Mingyao Ma, Davina Ngoi Wah Lie, Anthony Ting Pong Chan, Cheuk Chun Szeto, Kit Chung Jack Ng, Siu Fai Cheung, Tak Tai Andrew Yue, Ka Shun Samuel Fung, Hon Tang, Ka Fai Yim, Wai Ping Law, Yick Hei Wong, Chi Kwan Darwin Lam, Sze Ho Sunny Wong, Carmelita Marcantoni, Roberta Aliotta, Francesca Deodato, Gemma Patella, Nicolino Comi, Caterina Vita, Nazareno Carullo, Davide Bolignano, Michela Musolino, Matias Trillini, Norberto Perico, Giuseppe Remuzzi, Erica Daina, Luigi Biancone, Loredana Colla, Manuel Burdese, Chiara Cogno, Elena Boaglio, Isabella Abbasciano, Carlotta Federica Zizzi, Paolo Randone, Pietro Napodano, Anna Ricchiuto, Matthias Cassia, Simone Accarino, Mario Cozzolino, Rocco Baccaro, Stefano Costanzi, Federica Di Maio, Maria Arena, Federica Urciuolo, Sara Vigano, Andrea Cavalli, Monica Limardo, Monica Bordoli, Serena Ponti, Selena Longhi, Andrea Solazzo, Francesco Giaroni, Gabriele Donati, Massimo Torreggiani, Davide Catucci, Marco Colucci, Vittoria Esposito, Ciro Esposito, Loreto Gesualdo, Flavia Capaccio, Emma Diletta Stea, Carmen Sivo, Francesca Annese, Federica Papadia, Piergiorgio Messa, Mirco Belingheri, Patrizia Passerini, Silvia Malvica, Alvita Vickiene, Urte Zakauskiene, Egle Asakiene, Inga Arune Bumblyte', Asta Stankuviene, Lina Santockiene, Ashik Hayat, Allister Williams, Peter Sizeland, Eddie Tan, Gerald Waters, Lai Wan Chan, Andrew Henderson, Angus Turnbull, Andrew McNally, Annie Reynolds, Helen Pilmore, Ian Dittmer, Paul Manley, Elizabeth Stallworthy, Tze Goh, David Semple, Michael Collins, Elizabeth Curry, Jafar Ahmed, Thu Nguyen, Agata Winiarska, Justyna Zbrzezniak, Tomasz Stompor, Magdalena Krajewska, Hanna Augustyniak-Bartosik, Dorota Zielinska, Anna Jander, Malgorzata Stanczyk, Marcin Tkaczyk, Przemyslaw Miarka, Dariusz Aksamit, Piotr Jaskowski, Wladyslaw Sulowicz, Dominik Cieniawski, Julita Gontarek-Kacprzak, Elzbieta Felicjanczuk, Norbert Kwella, Bogna Kwella, Ewa Satora, João Carlos Fernandes, Ana Marta Gomes, Marina Reis, Daniela Lopes, Catarina Almeida, Helena Sá, Ana Carolina Figueiredo, Clara Pardinhas, Edgar Almeida, Mario Raimundo, Ana Cortesão Costa, Luis Pedro Falcao Goncalves, Sara Fernandes, Sónia Silva, Catarina Teixeira, Adriana Fernandes, Fernando Nolasco, Patricia Alves, Mario Gois, Nuno Fonseca, Ana Messias, Maria Menezes, Filipa Cardoso, Helena Sousa, Joana Marques, Rui Barata, Jose Antonio Lopes, Sofia Jorge, Joana Gameiro, Jose Nuno de Almeida Agapito Fonseca, Sara Goncalves, Ana Farinha, Patricia Valerio Santos, Ana Natario, Jose Carlos de Jesus Barreto, Catarina Abrantes, Elsa Sofia Quadrado Soares, Joana de Sousa Soares Felgueiras, Liliana Cunha, Lucia Parreira, Teresa Furtado, Alvaro Vaz, Kook-Hwan Oh, Hajeong Lee, Se Joong Kim, Jong Cheol Jeong, Yeong Hoon Kim, Yunmi Kim, Hyeong Cheon Park, Hoon Young Choi, Hyung Wook Kim, Moon Hyoung Lee, Songuk Yoon, Kyu-Beck Lee, YoungYoul Hyun, Tae-Hyun Yoo, Seung Hyeok Han, Jung Tak Park, Sunggyun Kim, Young Rim Song, Jwa-Kyung Kim, Hyung-seok Lee, Narae Joo, JungEun Lee, Hye Ryoun Jang, Junseok Jeon, Wookyung Chung, HyunHee Lee, Jae Hyun Chang, Ka Yeong Chun, Ji Yong Jung, Han Ro, Aejin Kim, Sang-Kyung Jo, Jihyun Yang, Myung-Gyu Kim, SeWon Oh, Caridad Martinez Villanueva, Ana Vilar Gimeno, Gustavo Andres Useche Bonilla, Esther Tamarit, Antonio Galan Serrano, Eduardo Verde Moreno, Jose Luño Fernandez, Maria Angeles Goicoechea Diezhandino, Ursula Verdalles Guzman, Ana Perez de Jose, Alberto Ortiz Arduan, María Vanessa Pérez Gómez, Catalina Martín Cleary, Raul Fernandez Prado, Elena Goma, Jose Ballarin, Montserrat Diaz Encarnacion, Iara Da Silva Santos, Helena Marco Rusinol, Monica Furlano, Carlos Arias, Clara Barrios, Eva Rodriguez Garcia, Adriana Sierra Ochoa, Belen Vizcaino Castillo, Jonay Pantoja Perez, Mercedes Gonzalez Moya, Mari Sargsyan, Emma Calatayud Aristoy, Ana Avila Bernabeu, Leticia Perez Lluna, Tamara Malek Marin, Maria Antonia Munar Vila, Ivon Maritza Bobadilla Rico, Natalia Allende Burgos, Eduardo Gutierrez Martinez, Elena Gutierrez Solis, Angel Sevillano, Evangelina Merida Herrero, Josep Miquel Blasco Pelicano, Lida Maria Rodas Marin, Luis F Quintana, Maria Antonieta Azancot Rivero, Natalia Ramos Terrades, Clara Garcia Carro, Irene Agraz Pamplona, Mercedes Salgueira Lazo, Francisco de la Prada Alvarez, Fabiola Alonso Garcia, Wenceslao Adrian Aguilera Morales, Salia Virxinia Pol Heres, Angel Forcen, Eduardo Parra Moncasi, Cristina Medrano Villarroya, Alejandro Soria Villen, Olga Gracia Garcia, Mercedes Velo Plaza, Maria Dolores Sánchez de la Nieta, Marta Calvo Arevalo, Antolina Moreno, Secundino Cigarran Guldris, Manuel Pereira de Vicente, Bang-Gee Hsu, Chih-Hsien Wang, Cheng-Hsu Chen, Tung-Min Yu, Ming-Ju Wu, Shang-Feng Tsai, Chia-Tien Hsu, Hsien-Fu Chiu, Kang-Ju Chou, Hua-Chang Fang, Po-Tsang Lee, Hsin-Yu Chen, Chien-Liang Chen, Chien-Wei Huang, Shih-Hsiang Ou, Tzung-Yo Ho, Chih-Yang Hsu, Ming-Shan Chang, Yen-Ling Chiu, Yu-Sen Peng, Kai-Hsiang Shu, Szu-Yu Pan, Shih-Ping Hsu, Ju-Yeh Yang, Mei-Fen Pai, Po-Yu Tseng, Hon-Yen Wu, Wan-Chuan Tsai, Kuei-Ting Tung, Hung-Yuan Chen, Hung-Chun Chen, Mei-Chuan Kuo, Daw-Yang Hwang, Yi-Wen Chiu, Chi-Chih Hung, Hung-Tien Kuo, Jer-Chia Tsai, Kieran McCafferty, Suzanne Forbes, Indranil Dasgupta, Mark Thomas, Amar Mahdi, Bamidele Ajayi, Paramit Chowdhury, Theodoros Kasimatis, Dimitrios Moutzouris, Caroline Dudreuilh, Rishi Pruthi, Nick Mansfield, Gabriel Doctor, Sapna Shah, Sui Kon, Priscilla Smith, Patrick Hamilton, Durga Kanigicherla, Omar Sherin Ibrahim Ragy, Bassam Alchi, Oliver Flossmann, Farid Ghalli, Sarah Lawman, Smeeta Sinha, Constantina Chrysochou, Chukwuma Chukwu, Aine Maire De Bhailis, Saif Al Chalabi, Amy Hudson, Arun Gopu, Olivia Wickens, Joshua Storrar, Mona Wahba, Nathan Lorde, Mohammad Rony, Sian Griffin, Farah Latif, Mohammad Ali, Louise DaSilva, Jonathan Ayling-Smith, Eamon Mahdi, Lisa Willcocks, Rachel Jones, Chee Kay Cheung, Haresh Selvaskandan, Dan Pugh, Matthew Sayer, Neeraj Dhaun, Fiona Chapman, Patrick Mark, Colin Geddes, Emily McQuarrie, Rajan Patel, Laurence Solomon, Arvind Ponnusamy, Adam Morris, Pedro Okoh, Lauren Floyd, Ajay Dhaygude, Janson Leung, Christopher Goldsmith, Bhavna Pandya, Didem Tez, Ashraf Mikhail, Karen Brown, Thomas Bucknall, Mark Lambie, Roderick Comunale, Donald Brandon, Stacy Martinez, Amanda Hall, Amy Henderson, Aaron Fearday, Nicole Douthit, Brian Snow, Arnold Silva, Cathylee Sly, Christopher Keller, Robert Davidson, Jerry Meng, Robert Haws, Siddhartha Kattamanchi, Javad Mojarrab, Unnikrishnan Pillai, Richard Lafayette, Michelle O'Shaughnessy, Fahameedah Kamal, Kshama Mehta, Bruce Baker, Mario Ruiz, Praveena Jyothinagaram, Usha Peri, William Paxton, James Tumlin, Kerri McGreal, Ellen McCarthy, Cassandra Kimber, Archana Gautam, Kassem Khalil, Viet Nguyen, Raffi Minasian, Dariush Arfaania, Sam Daneshvari, Michel Zakari, Artashes Patrikyan, Rouzbeh Afsari, Christine Ayvazyan, Faisal Fakih, Mark Lagatta, Alfred Rodriguez, Jorge Enrique Monroy Avella, Ramachandra Patak, Jigar Kadakia, Gerald Appel, Wooin Ahn, Bradley Nelson, Allyson Medina, Syeda Ahmad, Yonatan Peleg, Nisha Clement, Ian Chiu, Elizabeth Hendren, Andrew Bomback, Pietro Canetta, Bruce Spinowitz, Chaim Charytan, Nishita Parikh, Sheng Kuo, Ritesh Raichoudhury, Mirela Dobre, Lavinia Negrea, Aparna Padiyar, Arksarapuk Jittirat, Nishigandha Pradhan, Ranjit Dhelaria, Saravanan Balamuthusamy, Machaiah Madhrira, Thomas Powell, Howard Lifland, Asha Bailey, Sarah Ashley Ford Sightler, Meera Patel Suthar, Heather Green, Samir Parikh, Isabelle Ayoub, Salem Almaani, Gabriel Contreras, Alessia Fornoni, Yelena Drexler, Abdallah Geara, Brittany Sheridan, Gaia Coppock, Jonathan Hogan, Carlos Gonzalez, Shamik Bhadra, Pradip Chowdhury, Kay Kyaw, May Tan, Lathika Raakesh, Elder Mendoza, Veronica Viramontes, Asghar Chaudhry, Juan Carbonell, Rajdeep Gadh, Victor Fernandez, Mohamad Kassem, Radu Jacob, Karen Wilder, Britt Newsome, Kathryn Klamm, Irina Suyumova, Laura Ann Kooienga, Catherine Janko, Dana Rizk, Bruce Julian, Dawn Caster, Erika Perez, Gunjan Garg, Nayan Gowda, Suneel Udani, Sreedhar Mandayam, Biruh Workeneh, Ali Assefi, Barbara Greco, Michael Germain, Jusmin Patel, Sarah Quinn, James Sullivan, Jeffrey Glaze, Phillip Madonia, Kellyn McMahon, Harold Giles, Sharon Adler, and Tiane Dai
Subjects: General Medicine
Published: 2023

15. Validating a Computable Phenotype for Nephrotic Syndrome in Children and Adults Using PCORnet Data

Author: Cheryl L. Tran, Dorota M Marchel, Isabelle Ayoub, Michelle R. Denburg, Jessica Greco, Michael E. Matheny, Laura H. Mariani, Hailey Desmond, Andrea L. Oliverio, Chad Dorn, Debbie S. Gipson, Salem Almaani, Jonathan P. Troost, and Susan F. Massengill
Subjects: Male, Nephrology, medicine.medical_specialty, Pediatrics, Nephrotic Syndrome, Comparative effectiveness research, Lupus nephritis, International Classification of Diseases, Internal medicine, medicine, Electronic Health Records, Humans, Natural Language Processing, business.industry, General Medicine, medicine.disease, United States, Clinical trial, Phenotype, Editorial, Sample size determination, Female, Observational study, F1 score, business, Nephrotic syndrome
Abstract: Background: Primary nephrotic syndromes are rare diseases which impedes adequate sample size for observational patient-oriented research and clinical trial enrollment. A computable phenotype may be powerful in identifying patients with these diseases for research across multiple institutions. Methods: A comprehensive algorithm of inclusion and exclusion ICD-9 and ICD-10 codes to identify patients with primary nephrotic syndrome was developed. The algorithm was executed against the PCORnet® CDM at 3 institutions from Jan 1, 2009 to Jan 1, 2018, where a random selection of 50 cases and 50 non-cases (individuals not meeting case criteria seen within the same calendar year and within five years of age of a case) were reviewed by a nephrologist, for a total of 150 cases and 150 non-cases reviewed. The classification accuracy (sensitivity, specificity, positive and negative predictive value, F1 score) of the computable phenotype was determined. Results: The algorithm identified a total of 2,708 patients with nephrotic syndrome from 4,305,092 distinct patients in the CDM at all sites from 2009-2018. For all sites, the sensitivity, specificity, and area under the curve of the algorithm were 99% (95% CI: 97-99%), 79% (95% CI: 74-85%), and 0.9 (0.84-0.97), respectively. The most common causes of false positive classification were secondary FSGS (9/39) and lupus nephritis (9/39). Conclusion: This computable phenotype had good classification in identifying both children and adults with primary nephrotic syndrome utilizing only ICD-9 and ICD-10 codes, which are available across institutions in the United States. This may facilitate future screening and enrollment for research studies and enable comparative effectiveness research. Further refinements to the algorithm including use of laboratory data or addition of natural language processing may help better distinguish primary and secondary causes of nephrotic syndrome.
Published: 2021

16. ANCA Vasculitis Induction Management During the COVID-19 Pandemic

Author: Jennifer Scott, Adam D. Morris, Andreas Kronbichler, Vimal K. Derebail, Lauren Floyd, Mark A. Little, Stephen P. McAdoo, Philipp Gauckler, Silke R. Brix, Maria Prendecki, Tingting Li, Isabelle Ayoub, Sam Kant, Vladimir Tesar, Caroline J. Poulton, Antonio Salas, Ulf Schönermarck, Ajay Dhaygude, Manish K. Saha, Vojtech Kratky, Zdenka Hruskova, Purva Sharma, Duvuru Geetha, and Philip Seo
Subjects: 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), business.industry, Anca vasculitis, viruses, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), induction therapy, COVID pandemic, ANCA vasculitis, Virology, Nephrology, Induction therapy, Pandemic, Research Letter, Medicine, business
Abstract: As the severe acute respiratory syndrome coronavirus 2 pandemic evolved and became a global health threat, the safety of immunosuppression in antineutrophil cytoplasmic antibody-associated vasculitis (AAV) became of utmost important for clinicians and patients. Although timely initiation of immunosuppressive therapy is critical to quell the acute inflammation and prevent AAV-associated mortality and morbidity, concerns for increased susceptibility to Coronavirus Disease 2019 (COVID-19), delayed viral clearance, and decreased humoral response to infection led to speculation about modification in induction therapy practices may be deployed by physicians caring for patients with AAV. This international retrospective cohort study investigated the influence of the COVID-19 pandemic on AAV induction therapy and patient outcomes in different parts of the world by studying differences in treatment regimens in the United States, United Kingdom, and Europe.
Published: 2021

17. Glomerular crescents, IgA-deposits, ANCA, infection – unravelling the diagnostic conundrum

Author: Mineaki Kitamura, Salem Almaani, Bindu Challa, Mohankumar Doraiswamy, Isabelle Ayoub, Laura Biederman, Samir V. Parikh, Ana Molovic-Kokovic, Jason Benedict, Nilesh Mhaskar, Zeid Khitan, Sergey V. Brodsky, Tibor Nadasdy, and Anjali A Satoskar
Abstract: IntroductionGlomerulonephritis (GN) with crescents and IgA deposits on kidney biopsy poses a frequent diagnostic and therapeutic dilemma because of multiple possibilities.MethodsNative kidney biopsies showing IgA deposition and crescents (excluding lupus nephritis) were identified from our biopsy archives between January 2010 and December 2021. Detailed clinico-pathologic features were assessed. One-year clinical follow-up on a subset of cases was performed.ResultsA total of 285 cases were identified and these clustered into IgA nephropathy (IgAN, n=108), Staphylococcus or other infection-associated-GN (SAGN/IRGN, n=46), and anti-neutrophil cytoplasmic antibody associated-GN (ANCA-GN, n=24) based on constellation of clinico-pathologic features, but 101 cases (Group X) could not be definitively differentiated. The reasons have been elucidated, most important being atypical combination of clinico-pathologic features and lack of definitive evidence of active infection. Follow-up (on 72/101 cases), revealed that clinicians’ working diagnosis was IgAN in 42%, SAGN/IRGN in 24%, ANCA-GN in 24%, and others in 7% of the cases, but treatment approach varied from supportive/antibiotics to immunosuppression in each subgroup. Comparing these cases as “received immunosuppression” versus “no-immunosuppression”, only two features - C3-dominant staining; and possibility of recent infection differed (higher in the no-immunosuppression group [pConclusionDiagnostic overlap may remain unresolved in a substantial number of kidney biopsies with glomerular crescents and IgA deposits. A case-by-case approach, appropriate antibiotics if infection is ongoing, and consideration for cautious immunosuppressive treatment for progressive renal dysfunction may be needed for best chance of renal recovery.
Published: 2022

18. Results from part A of the multi-center, double-blind, randomized, placebo-controlled NefIgArd trial, which evaluated targeted-release formulation of budesonide for the treatment of primary immunoglobulin A nephropathy

Author: Jonathan Barratt, Richard Lafayette, Jens Kristensen, Andrew Stone, Daniel Cattran, Jürgen Floege, Vladimir Tesar, Hernán Trimarchi, Hong Zhang, Necmi Eren, Alexander Paliege, Brad H. Rovin, Guillermo Fragale, Alejandra Karl, Patricia Losisolo, Ivan Gonzalez Hoyos, Mauro Guillermo Lampo, Matias Monkowski, Jorge De La Fuente, Magdalena Alvarez, Daniela Stoppa, Carlos Chiurchiu, Pablo Antonio Novoa, Marcelo Orias, Maria Belen Barron, Ana Giotto, Mariano Arriola, Evelin Cassini, Rafael Maldonado, Maria Paula Dionisi, Jessica Ryan, Nigel Toussaint, Grant Luxton, Chen Au Peh, Vicki Levidiotis, Ross Francis, Richard Phoon, Elena Fedosiuk, Dmitry Toropilov, Ruslan Yakubtsevich, Elena Mikhailova, Christophe Bovy, Nathalie Demoulin, Jean-Michel Hougardy, Bart Maes, Marijn Speeckaert, Louis-Philippe Laurin, Sean Barbour, Melanie Masse, Michelle Hladunewich, Heather Reich, Serge Cournoyer, Karthik Tennankore, Jicheng Lv, Zhangsuo Liu, Caili Wang, Shaomei Li, Qun Luo, Zhaohui Ni, Tiekun Yan, Ping Fu, Hong Cheng, Bicheng Liu, Wanhong Lu, Jianqin Wang, Qinkai Chen, DeGuang Wang, Zuying Xiong, Menghua Chen, Yan Xu, Jiali Wei, Pearl Pai, Lianhua Chen, Jitka Rehorova, Dita Maixnerova, Roman Safranek, Ivan Rychlik, Miroslav Hruby, Satu Makela, Kati Vaaraniemi, Fernanda Ortiz, Eric Alamartine, Maite Daroux, Claire Cartery, Francois Vrtovsnik, Jean-Emmanuel Serre, Eleni Stamellou, Volker Vielhauer, Christian Hugo, Klemens Budde, Britta Otte, Martin Nitschke, Evangelia Ntounousi, Ioannis Boletis, Aikaterini Papagianni, Dimitrios Goumenos, Konstantinos Stylianou, Synodi Zermpala, Ciro Esposito, Mario Gennaro Cozzolino, Sara Maria Viganò, Loreto Gesualdo, Michal Nowicki, Tomasz Stompor, Ilona Kurnatowska, Sung Gyun Kim, Yong-Lim Kim, Ki-Ryang Na, Dong Ki Kim, Su-Hyun Kim, Luis Quintana Porras, Eva Rodriguez Garcia, Irene Agraz Pamplona, Alfons Segarra, Marian Goicoechea, Bengt Fellstrom, Sigrid Lundberg, Peter Hemmingsson, Gregor Guron, Anna Sandell, Cheng-Hsu Chen, Bulent Tokgoz, Soner Duman, Mehmet Riza Altiparmak, Metin Ergul, Peter Maxwell, Patrick Mark, Kieran McCafferty, Arif Khwaja, Chee Kay Cheung, Matthew Hall, Albert Power, Durga Kanigicherla, Richard Baker, Jim Moriarty, Amr Mohamed, Joseph Aiello, Pietro Canetta, Isabelle Ayoub, Derrick Robinson, Surabhi Thakar, Amy Mottl, Isaac Sachmechi, Bernard Fischbach, Harmeet Singh, Jeffrey Mulhern, Fahmeedah Kamal, Douglas Linfert, Dana Rizk, Shikha Wadhwani, Menaka Sarav, Kirk Campbell, Gaia Coppock, Randy Luciano, John Sedor, Rupali Avasare, Wai Lang Lau, Zermpala, Synodi, Esposito, Ciro, Cozzolino, Mario Gennaro, Viganò, Sara Maria, Gesualdo, Loreto, Nowicki, Michal, Stompor, Tomasz, Kurnatowska, Ilona, Kim, Sung Gyun, Kim, Yong-Lim, Na, Ki-Ryang, Kim, Dong Ki, Kim, Su-Hyun, Porras, Luis Quintana, Garcia, Eva Rodriguez, Pamplona, Irene Agraz, Segarra, Alfons, Goicoechea, Marian, Fellstrom, Bengt, Lundberg, Sigrid, Hemmingsson, Peter, Guron, Gregor, Sandell, Anna, Chen, Cheng-Hsu, Tokgoz, Bulent, Duman, Soner, Altiparmak, Mehmet Riza, Ergul, Metin, Maxwell, Peter, Mark, Patrick, Fragale, Guillermo, McCafferty, Kieran, Khwaja, Arif, Cheung, Chee Kay, Hall, Matthew, Power, Albert, Kanigicherla, Durga, Baker, Richard, Moriarty, Jim, Mohamed, Amr, Aiello, Joseph, Karl, Alejandra, Canetta, Pietro, Ayoub, Isabelle, Robinson, Derrick, Thakar, Surabhi, Mottl, Amy, Sachmechi, Isaac, Fischbach, Bernard, Singh, Harmeet, Mulhern, Jeffrey, Kamal, Fahmeedah, Losisolo, Patricia, Linfert, Douglas, Rizk, Dana, Wadhwani, Shikha, Sarav, Menaka, Campbell, Kirk, Coppock, Gaia, Luciano, Randy, Sedor, John, Avasare, Rupali, Lau, Wai Lang, Trimarchi, Hernán, Hoyos, Ivan Gonzalez, Lampo, Mauro Guillermo, Monkowski, Matias, De La Fuente, Jorge, Alvarez, Magdalena, Stoppa, Daniela, Chiurchiu, Carlos, Novoa, Pablo Antonio, Orias, Marcelo, Barron, Maria Belen, Giotto, Ana, Arriola, Mariano, Cassini, Evelin, Maldonado, Rafael, Dionisi, Maria Paula, Ryan, Jessica, Toussaint, Nigel, Luxton, Grant, Peh, Chen Au, Levidiotis, Vicki, Francis, Ross, Phoon, Richard, Fedosiuk, Elena, Toropilov, Dmitry, Yakubtsevich, Ruslan, Mikhailova, Elena, Bovy, Christophe, Demoulin, Nathalie, Hougardy, Jean-Michel, Maes, Bart, Speeckaert, Marijn, Laurin, Louis-Philippe, Barbour, Sean, Masse, Melanie, Hladunewich, Michelle, Reich, Heather, Cournoyer, Serge, Tennankore, Karthik, Lv, Jicheng, Liu, Zhangsuo, Wang, Caili, Li, Shaomei, Luo, Qun, Ni, Zhaohui, Yan, Tiekun, Fu, Ping, Cheng, Hong, Liu, Bicheng, Lu, Wanhong, Wang, Jianqin, Chen, Qinkai, Wang, DeGuang, Xiong, Zuying, Chen, Menghua, Xu, Yan, Wei, Jiali, Pai, Pearl, Chen, Lianhua, Rehorova, Jitka, Maixnerova, Dita, Safranek, Roman, Rychlik, Ivan, Hruby, Miroslav, Makela, Satu, Vaaraniemi, Kati, Ortiz, Fernanda, Alamartine, Eric, Daroux, Maite, Cartery, Claire, Vrtovsnik, Francois, Serre, Jean-Emmanuel, Stamellou, Eleni, Vielhauer, Volker, Hugo, Christian, Budde, Klemens, Otte, Britta, Nitschke, Martin, Ntounousi, Evangelia, Boletis, Ioannis, Papagianni, Aikaterini, Goumenos, Dimitrios, Stylianou, Konstantinos, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, and UCL - (SLuc) Service de néphrologie
Subjects: gut-associated lymphoid tissue, glucocorticoids, Nephrology, glomerular disease, IgA nephropathy
Abstract: Kidney international 103(2), 391-402 (2022). doi:10.1016/j.kint.2022.09.017, Published by Elsevier, New York, NY
Published: 2022

19. Daratumumab in Patients With Bortezomib-Refractory Proliferative Glomerulonephritis With Monoclonal Immunoglobulin Deposits

Author: Brad H. Rovin, Anjali A. Satoskar, Isabelle Ayoub, Nidhi Sharma, Naresh Bumma, Salem Almaani, Samir V. Parikh, and Yvonne Efebera
Subjects: Bortezomib, business.industry, Daratumumab, Monoclonal immunoglobulin, Glomerulonephritis, medicine.disease, daratumumab, MGRS, PGNMID, Refractory, Nephrology, Immunology, medicine, Research Letter, In patient, business, medicine.drug
Published: 2021

20. Evaluation of the Reproductive Care Provided to Adolescent Patients in Nephrology Clinics: A Pediatric Nephrology Research Consortium Study

Author: Tetyana L. Vasylyeva, Abigail Batson, Hilda Fernandez, Michelle M. O’Shaughnessy, William E. Smoyer, Noel Howard, Isabelle Ayoub, Monica L. Reynolds, Katherine Twombley, Shikha Wadhwani, Shyanne Page-Hefley, Jarcy Zee, Scott E. Wenderfer, Salem Almaani, and Michelle A. Hladunewich
Subjects: Nephrology, medicine.medical_specialty, Reproductive care, business.industry, Internal medicine, Family medicine, medicine, MEDLINE, Research Letter, Pediatric nephrology, business
Published: 2021

21. Establishing a Case for Anti-complement Therapy in Membranous Nephropathy

Author: Sergey V. Brodsky, John P. Shapiro, Xiaolan Lily Zhang, Salem Almaani, Michael L. Merchant, Anjali A. Satoskar, Cherri Bott, John Klein, Juan M. Mejia-Vilet, Daniel J. Birmingham, Brad H. Rovin, Samir V. Parikh, Lianbo Yu, Tibor Nadasdy, Sethu Madhavan, Huijuan Song, and Isabelle Ayoub
Subjects: complement protein, Pathology, medicine.medical_specialty, 030232 urology & nephrology, 030204 cardiovascular system & hematology, lcsh:RC870-923, 03 medical and health sciences, 0302 clinical medicine, Complement Receptor Type 1, Membranous nephropathy, Translational Research, medicine, Kidney, biology, business.industry, membranous nephropathy, proteomic analysis, lcsh:Diseases of the genitourinary system. Urology, medicine.disease, Complement system, medicine.anatomical_structure, Nephrology, Lectin pathway, biology.protein, Vitronectin, business, Complement membrane attack complex, Kidney disease
Abstract: Introduction Membranous nephropathy (MN) is a common cause of adult nephrotic syndrome that progresses to end-stage kidney disease in up to 40% of cases. It is an autoimmune disease characterized by glomerular subepithelial deposits containing IgG. In experimental MN, these deposits activate complement and cause kidney damage. The role of complement in human MN is less clearly defined. To address this, the current study focused on the role of complement in 2 independent primary (p) MN cohorts. Methods Glomeruli were isolated by laser capture microdissection and analyzed by mass spectrometry, focusing on complement proteins, from kidney biopsy specimens from a pMN cohort (n = 11) and from normal controls (n = 5). Immunohistological staining of kidney biopsy specimens for complement proteins was also done. In a second pMN cohort (n = 13), urine levels of Ba, C5a, and C5b-9 (membrane attack complex [MAC]) were measured. Results Mass spectrometry identified 8 complement pathway components (C1q, C3, C4, C5, C6, C7, C8, and C9) and 5 complement regulators (complement receptor type 1 [CR1], factor H [FH], FH-related protein 2 [FHR2], vitronectin, and clusterin). All complement levels were significantly higher in the MN groups than in the control group, except the level of CR1, which was lower. All pMN biopsy specimens showed negative or trace staining for C1q, positive staining for C3 and C4, and positive staining for at least 1 component of the lectin pathway. Urine Ba, C5a, and MAC were present in pMN, and their levels correlated (rBa,C5a = 0.87, rBa,MAC = 0.89, and rC5a,MAC = 0.97, P = .001 for each correlation). Conclusion Elevated glomerular levels of C3, C4, and components of MAC (C5b-9) and absent or decreased levels of the complement regulator CR1, along with increased levels of complement activation products in the urine, support the involvement of complement in the pathogenesis of MN. These data raise the possibility that anti-complement therapies may be effective in some forms of MN., Graphical abstract
Published: 2021

22. Diffuse 'background' monoclonal light chain staining on kidney biopsies in the absence of electron-dense deposits – putting it into perspective: A retrospective cohort study

Author: Anjali A. Satoskar, Sergey V. Brodsky, Ahlim Alsanani, Isabelle Ayoub, Abdullah Khan, Srinivas Devarakonda, Clarissa A. Cassol, Kiran Kandukurti, Jason Prosek, and Tibor Nadasdy
Subjects: Kidney, Pathology, medicine.medical_specialty, medicine.diagnostic_test, business.industry, Paraproteinemias, Retrospective cohort study, General Medicine, medicine.disease, Immunoglobulin light chain, Immunofluorescence, Staining, medicine.anatomical_structure, Nephrology, Monoclonal, Biopsy, medicine, Humans, Kidney Diseases, business, Retrospective Studies, Kidney disease
Abstract: Background Pathologic diagnosis of monoclonal gammopathy (MIg)-associated kidney disease requires specific morphologic and immunofluorescence (IF) findings with deposits on electron microscopy. We have encountered kidney biopsies showing only diffuse "background" monoclonal light chain staining, without characteristic morphologic or ultrastructural findings. Such staining is often overlooked if weak, or over-diagnosed as MIg-associated kidney disease if strong, causing dilemma over the need for immediate clone-directed therapy. We performed a clinicopathologic study to better understand its significance. Materials and methods Database search revealed 32 such cases over 12 years. Demographic, laboratory, and pathology data were retrieved along with a mean follow-up of 13 months. Results 15/32 (47%) patients did have active myeloma on hematologic testing (without myeloma casts) warranting immediate clone-directed therapy; but 11/32 (34%) did not develop active myeloma; 3/32 (9%) did not even have detectable paraprotein; 3/32 (9%) were lost to follow-up. Importantly, strong background light chain staining was seen even in some non-myeloma biopsies and conversely, weak staining was seen in some myeloma biopsies, complicating diagnosis. Conclusion It is important to recognize and document this finding in the biopsy report, but by itself, it should not be classified as MIg-associated kidney disease even in the face of strong staining intensity. A thorough hematologic work-up is critically important to unmask underlying active myeloma, which many patients may have. But equally important is to avoid inadvertent clone-directed therapy in patients who do not have active myeloma despite the background monoclonal staining. A protocol for periodic monitoring with hematologic and renal parameters to watch for possible malignant transformation is recommend for timely implementation of therapy to minimize renal damage.
Published: 2020

23. Hemophagocytic Lymphohistiocytosis, a Rare Presentation in Lupus Nephritis

Author: Isabelle Ayoub, Rima Kang, Nicholas L. Li, and Stacy P. Ardoin
Subjects: Autoimmune disease, endocrine system, Hemophagocytic lymphohistiocytosis, Hemophagocytic syndromes, business.industry, fungi, Lupus nephritis, Disease, medicine.disease, Nephrology, hemic and lymphatic diseases, Macrophage activation syndrome, Immunology, medicine, Immune disorder, Presentation (obstetrics), business, Nephrology Rounds, hormones, hormone substitutes, and hormone antagonists
Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare immune disorder that is life threatening if not promptly diagnosed and treated. Its identification, however, remains a diagnostic challenge for clinicians, given the large overlap in presenting symptoms with other conditions, including autoimmune disease and infection. Further adding to the complexity is that HLH encompasses 2 separate forms of disease (Figure1): primary or familial HLH (FHL), and secondary HLH (sHLH), which is often referred to as macrophage activation syndrome (MAS) when associated with rheumatological disease and which can occur in response to robust immunological activation. Open in a separate window Figure 1 Overview of the terminology of hemophagocytic syndromes.
Published: 2020

24. Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulopathy Network

Author: Dhruti P. Chen, Margaret E. Helmuth, Abigail R. Smith, Pietro A. Canetta, Isabelle Ayoub, Krzysztof Mucha, Mahmoud Kallash, Jeffrey B. Kopp, Rasheed Gbadegesin, Brenda W. Gillespie, Larry A. Greenbaum, Rulan S. Parekh, Tracy E. Hunley, C. John Sperati, David T. Selewski, Jason Kidd, Aftab Chishti, Kimberly Reidy, Amy K. Mottl, Debbie S. Gipson, Tarak Srivastava, Katherine E. Twombley, Wooin Ahn, Gerald Appel, Paul Appelbaum, Revekka Babayev, Andrew Bomback, Brenda Chan, Vivette Denise D’Agati, Samitri Dogra, Hilda Fernandez, Ali Gharavi, William Hines, Syed Ali Husain, Namrata Jain, Krzysztof Kiryluk, Fangming Lin, Maddalena Marasa, Glen Markowitz, Hila Milo Rasouly, Sumit Mohan, Nicola Mongera, Jordan Nestor, Thomas Nickolas, Jai Radhakrishnan, Maya Rao, Simone Sanna-Cherchi, Shayan Shirazian, Michael Barry Stokes, Natalie Uy, Anthony Valeri, Natalie Vena, Bartosz Foroncewicz, Barbara Moszczuk, Agnieszka Perkowska-Ptasińska, Gian Marco Ghiggeri, Francesca Lugani, Josephine Ambruzs, Helen Liapis, Rossana Baracco, Amrish Jain, Isa Ashoor, Diego Aviles, Sun-Young Ahn, Prasad Devarajan, Elif Erkan, Donna Claes, Hillarey Stone, Sherene Mason, Liliana Gomez-Mendez, Chia-shi Wang, Hong Yin, Yi Cai, Goebel Jens, Julia Steinke, Donald Weaver, Jerome Lane, Carl Cramer, Cindy Pan, Neil Paloian, Rajasree Sreedharan, Corinna Bowers, Mary Dreher, John Mahan, Samantha Sharpe, William Smoyer, Amira Al-Uzri, Sandra Iragorri, Myda Khalid, Craig Belsha, Joseph Alge, Michael Braun, A.C. Gomez, Scott Wenderfer, Tetyana Vasylyeva, Daniel Feig, Gabriel Cara Fuentes, Melisha Hannah, Carla Nester, Jon Klein, Chryso Katsoufis, Wacharee Seeherunvong, Michelle Rheault, Craig Wong, Nisha Mathews, John Barcia, Agnes Swiatecka-Urban, Sharon Bartosh, Vikas Dharnidharka, Joseph Gaut, Louis-Philippe Laurin, Virginie Royal, Anand Achanti, Milos Budisavljevic, Sally Self, Cybele Ghossein, Yonatan Peleg, Shikha Wadhwani, Salem Almaani, Tibor Nadasdy, null Samir, null Parikh, Brad Rovin, Anthony Chang, Huma Fatima, Bruce Julian, Jan Novak, Matthew Renfrow, Dana Rizk, Vimal Derebail, Ronald Falk, Keisha Gibson, Dorey Glenn, Susan Hogan, Koyal Jain, J. Charles Jennette, Caroline Poulton, Manish Kanti Saha, Agnes Fogo, Neil Sanghani, Selvaraj Muthusamy, Jeffrey Schelling, Jean Hou, Kevin Lemley, Warren Mika, Pierre Russo, Michelle Denburg, Amy Kogon, Kevin Meyers, Madhura Pradhan, Raed Bou Matar, John O’Toole, John Sedor, Christine Sethna, Suzanne Vento, Mohamed Atta, Serena Bagnasco, Alicia Neu, Sharon Adler, Tiane Dai, Ram Dukkipati, Fernando Fervenza, Sanjeev Sethi, Frederick Kaskel, Kaye Brathwaite, Joseph Weisstuch, Ming Wu, Olga Zhdanova, Jurgen Heymann, Meryl Waldman, Cheryl Winkler, Katherine Tuttle, Jill Krissberg, Richard Lafayette, Kamal Fahmeedah, Elizabeth Talley, Michelle Hladunewich, Carmen Avila-Casado, Daniel Cattran, Reich Heather, Philip Boll, Yelena Drexler, Alessia Fornoni, Patrick Gipson, Jeffrey Hodgin, Andrea Oliverio, Jon Hogan, Lawrence Holzman, Matthew Palmer, Gaia Coppock, Blaise Abromovitz, Michael Mortiz, Charles Alpers, J. Ashley Jefferson, Elizabeth Brown, Kamal Sambandam, Bethany Roehm, Bruce Robinson, Cynthia Nast, Laura Barisoni, Matthias Kretzler, Laura Mariani, and Lisa M. Guay-Woodford
Subjects: Nephrology
Abstract: Adolescent- and adult-onset disease minimal change disease (MCD) may have a clinical course distinct from childhood-onset disease. We characterized the course of children and adults with MCD in the Cure Glomerulopathy Network (CureGN) and assessed predictors of rituximab response.Prospective, multicenter, observational study.CureGN participants with proven MCD on biopsy.Age at disease onset. Initiation of RAAS blockade and immunosuppression including rituximab during the study period.Relapse and remission, change in eGFR and kidney failure.Remission and relapse probabilities are estimated using Kaplan-Meier curves and gap time recurrent event models. Linear regression models were used for the outcome of change in estimated glomerular filtration rate (eGFR). Cox proportional hazards models were used to estimate the association between rituximab administration and remission.304 childhood (≤12 years old), 49 adolescent (13-17 years old), and 201 adult onset (≥18 years) participants were included with 2.7-3.2 years of follow-up after enrollment. Children had longer time to biopsy (238 days vs. 23 in adolescents, and 36 in adults, p0.001) and were more likely to have received therapy prior to biopsy. Children were more likely to be treated with immunosuppression but not RAAS blockade. The rate of relapse was higher in childhood-onset versus adult-onset participants (hazard ratio (HR) 1.69 (95% confidence interval (CI) 1.29-2.21)). The probability of remission was also higher in childhood-onset disease (HR 1.33 (95%CI 1.02-1.72)). eGFR loss in all groups was minimal. Children were more likely to remit after rituximab than adolescents and adults (adjusted HR 2.1, p=0.003). Across all groups, glucocorticoid-sensitivity was associated with a greater likelihood of achieving complete remission after rituximab (adjusted HR 2.62, p=0.002).CureGN was limited to biopsy-proven disease. Comparisons of childhood to non-childhood cases of MCD may be subject to selection bias, given that childhood cases who are biopsied may be limited to those patients who are least responsive to initial therapy.Among patients with MCD who underwent kidney biopsy, there were differences in the course (relapse and remission) of childhood-onset compared to adolescent and adult-onset disease, as well as rituximab response.
Published: 2022

25. Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

Author: Elisa Delbarba, Maddalena Marasa, Pietro A. Canetta, Stacy E. Piva, Debanjana Chatterjee, Byum Hee Kil, Xueru Mu, Keisha L. Gibson, Michelle A. Hladunewich, Jonathan J. Hogan, Bruce A. Julian, Jason M. Kidd, Louis-Philippe Laurin, Patrick H. Nachman, Michelle N. Rheault, Dana V. Rizk, Neil S. Sanghani, Howard Trachtman, Scott E. Wenderfer, Ali G. Gharavi, Andrew S. Bomback, Wooin Ahn, Gerald B. Appel, Revekka Babayev, Ibrahim Batal, Eric Brown, Eric S. Campenot, Pietro Canetta, Brenda Chan, Vivette D. D’Agati, Hilda Fernandez, Bartosz Foroncewicz, Gian Marco Ghiggeri, William H. Hines, Namrata G. Jain, Krzysztof Kiryluk, Wai L. Lau, Fangming Lin, Francesca Lugani, Glen Markowitz, Sumit Mohan, Krzysztof Mucha, Thomas L. Nickolas, Stacy Piva, Jai Radhakrishnan, Maya K. Rao, Simone Sanna-Cherchi, Dominick Santoriello, Michael B. Stokes, Natalie Yu, Anthony M. Valeri, Ronald Zviti, Larry A. Greenbaum, William E. Smoyer, Amira Al-Uzri, Isa Ashoor, Diego Aviles, Rossana Baracco, John Barcia, Sharon Bartosh, Craig Belsha, Corinna Bowers, Michael C. Braun, Aftab Chishti, Donna Claes, Carl Cramer, Keefe Davis, Elif Erkan, Daniel Feig, Michael Freundlich, Rasheed Gbadegesin, Melisha Hanna, Guillermo Hidalgo, Tracy E. Hunley, Amrish Jain, Mahmoud Kallash, Myda Khalid, Jon B. Klein, Jerome C. Lane, John Mahan, Nisha Mathews, Carla Nester, Cynthia Pan, Larry Patterson, Hiren Patel, Adelaide Revell, Cynthia Silva, Rajasree Sreedharan, Tarak Srivastava, Julia Steinke, Katherine Twombley, Tetyana L. Vasylyeva, Donald J. Weaver, Craig S. Wong, Salem Almaani, Isabelle Ayoub, Milos Budisavljevic, Vimal Derebail, Huma Fatima, Ronald Falk, Agnes Fogo, Todd Gehr, Keisha Gibson, Dorey Glenn, Raymond Harris, Susan Hogan, Koyal Jain, J. Charles Jennette, Bruce Julian, Jason Kidd, H. Davis Massey, Amy Mottl, Patrick Nachman, Tibor Nadasdy, Jan Novak, Samir Parikh, Vincent Pichette, Caroline Poulton, Thomas Brian Powell, Matthew Renfrow, Dana Rizk, Brad Rovin, Virginie Royal, Manish Saha, Neil Sanghani, Sally Self, Sharon Adler, Charles Alpers, Raed Bou Matar, Elizabeth Brown, Daniel Cattran, Michael Choi, Katherine M. Dell, Ram Dukkipati, Fernando C. Fervenza, Alessia Fornoni, Crystal Gadegbeku, Patrick Gipson, Leah Hasely, Sangeeta Hingorani, Michelle Hladunewich, Jonathan Hogan, Lawrence B. Holzman, J. Ashley Jefferson, Kenar Jhaveri, Duncan B. Johnstone, Frederick Kaskel, Amy Kogan, Jeffrey Kopp, Richard Lafayette, Kevin V. Lemley, Laura Malaga-Dieguez, Kevin Meyers, Alicia Neu, Michelle Marie O’Shaughnessy, John F. O’Toole, Rulan Parekh, Heather Reich, Kimberly Reidy, Helbert Rondon, Kamalanathan K. Sambandam, John R. Sedor, David T. Selewski, Christine B. Sethna, Jeffrey Schelling, John C. Sperati, Agnes Swiatecka-Urban, Katherine R. Tuttle, Joseph Weisstuch, Suzanne Vento, Olga Zhdanova, Brenda Gillespie, Debbie S. Gipson, Peg Hill-Callahan, Margaret Helmuth, Emily Herreshoff, Matthias Kretzler, Chrysta Lienczewski, Sarah Mansfield, Laura Mariani, Cynthia C. Nast, Bruce M. Robinson, Jonathan Troost, Matthew Wladkowski, Jarcy Zee, Dawn Zinsser, and Lisa M. Guay-Woodford
Subjects: medicine.medical_specialty, glomerulonephropathy, glomerular disease, 030232 urology & nephrology, Disease, 030204 cardiovascular system & hematology, Nephropathy, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Focal segmental glomerulosclerosis, Membranous nephropathy, Clinical Research, Internal medicine, Biopsy, medicine, Minimal change disease, focal segmental glomerulosclerosis, Creatinine, medicine.diagnostic_test, business.industry, membranous nephropathy, IgA nephropathy, medicine.disease, minimal change disease, chemistry, Nephrology, Cohort, business
Abstract: Introduction Glomerular diseases are characterized by variable disease activity over many years. We aimed to analyze the relationship between clinical disease activity and duration of glomerular disease. Methods Disease activity in adults with chronic minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy (IgAN; first diagnostic biopsy >5 years before enrollment; Of Longstanding Disease [OLD] cohort, n = 256) followed at Columbia University Medical Center (CUMC), was compared with disease activity of an internal and external cohort of patients with first diagnostic biopsy, Graphical abstract
Published: 2020

26. The Use of Serological Tests in the Care of Patients with Lupus Nephritis

Author: Brad H. Rovin and Isabelle Ayoub
Subjects: Adult, Transplantation, medicine.medical_specialty, Epidemiology, business.industry, Lupus nephritis, Critical Care and Intensive Care Medicine, medicine.disease, Dermatology, Lupus Nephritis, Serology, Nephrology, medicine, Humans, Female, Serologic Tests, business, Kidney Case Conference: How I Treat
Published: 2022

27. Racial and Ethnic Disparities in Acute Care Utilization Among Patients With Glomerular Disease

Author: Jill R. Krissberg, Michelle M. O’Shaughnessy, Abigail R. Smith, Margaret E. Helmuth, Salem Almaani, Diego H. Aviles, Kaye E. Brathwaite, Yi Cai, Daniel Cattran, Rasheed Gbadegesin, Dorey A. Glenn, Larry A. Greenbaum, Sandra Iragorri, Koyal Jain, Myda Khalid, Jason Kidd, Jeffrey Kopp, Richard Lafayette, Jerome C. Lane, Francesca Lugani, Jordan G. Nestor, Rulan S. Parekh, Kimberly Reidy, David T. Selewski, Christine B. Sethna, C. John Sperati, Katherine Tuttle, Katherine Twombley, Tetyana L. Vasylyeva, Donald J. Weaver, Scott E. Wenderfer, Keisha Gibson, Wooin Ahn, Gerald Appel, Paul Appelbaum, Revekka Babayev, Andrew Bomback, Eric Brown, Pietro Canetta, Lucrezia Carlassara, Brenda Chan, Vivette Denise D’Agati, Samitri Dogra, Hilda Fernandez, Ali Gharavi, William Hines, Syed Ali Husain, Krzysztof Kiryluk, Fangming Lin, Maddalena Marasa, Glen Markowitz, Hila Milo Rasouly, Sumit Mohan, Nicola Mongera, Thomas Nickolas, Jai Radhakrishnan, Maya Rao, Simone Sanna-Cherchi, Shayan Shirazian, Michael Barry Stokes, Natalie Uy, Anthony Valeri, Natalie Vena, Bartosz Foroncewicz, Barbara Moszczuk, Krzysztof Mucha, Agnieszka Perkowska-Ptasińska, Gian Marco Ghiggeri, Josephine Ambruzs, Helen Liapis, Rossana Baracco, Amrish Jain, Isa Ashoor, Tarak Srivastava, Sun-Young Ahn, Prasad Devarajan, Elif Erkan, Donna Claes, Hillarey Stone, Sherene Mason, Cynthia Silva, Liliana Gomez-Mendez, Chia-shi Wang, Hong (Julie) Yin, Goebel Jens, Julia Steinke, Carl Cramer, Cindy Pan, Rajasree Sreedharan, Corinna Bowers, Mary Dreher, Mahmoud Kallash, John Mahan, Samantha Sharpe, William Smoyer, Amira Al-Uzri, Craig Belsha, Michael Braun, A.C. Gomez, Daniel Feig, Gabriel Cara Fuentes, Melisha Hannah, Carla Nester, Aftab Chishti, Jon Klein, Chryso Katsoufis, Wacharee Seeherunvong, Michelle Rheault, Craig Wong, Nisha Mathews, John Barcia, Agnes Swiatecka-Urban, Sharon Bartosh, Tracy Hunley, Vikas Dharnidharka, Joseph Gaut, Louis-Philippe Laurin, Virginie Royal, Anand Achanti, Milos Budisavljevic, Sally Self, Cybele Ghossein, Shikha Wadhwani, Isabelle Ayoub, Tibor Nadasdy, Samir Parikh, Brad Rovin, Anthony Chang, Huma Fatima, Jan Novak, Matthew Renfrow, Dana Rizk, Dhruti Chen, Vimal Derebail, Ronald Falk, Susan Hogan, J. Charles Jennette, Amy Mottl, Caroline Poulton, Manish Kanti Saha, Agnes Fogo, Neil Sanghani, Hugh Massey, Selvaraj Muthusamy, Santhi Ganesan, Agustin Gonzalez-Vicente, Jeffrey Schelling, Jean Hou, Kevin Lemley, Warren Mika, Pierre Russo, Michelle Denburg, Amy Kogon, Kevin Meyers, Madhura Pradhan, Raed Bou Matar, John O’Toole, John Sedor, Serena Bagnasco, Alicia Neu, Sharon Adler, Tiane Dai, Ram Dukkipati, Fernando Fervenza, Sanjeev Sethi, Frederick Kaskel, Suzanne Vento, Joseph Weisstuch, Ming Wu, Olga Zhdanova, Jurgen Heymann, Meryl Waldman, Cheryl Winkler, Michelle Hladunewich, Carmen Avila-Casado, Reich Heather, Philip Boll, Yelena Drexler, Alessia Fornoni, Patrick Gipson, Jeffrey Hodgin, Andrew Oliverio, Jon Hogan, Lawrence Holzman, Matthew Palmer, Blaise Abromovitz, Michael Mortiz, Charles Alpers, J. Ashley Jefferson, Elizabeth Brown, Kamal Sambandam, Bruce Robinson, Cynthia Nast, Laura Barisoni, Brenda Gillespie, Deb Gipson, Maggie Hicken, Matthias Kretzler, Laura Mariani, and Lisa M. Guay-Woodford
Subjects: Nephrology
Abstract: The effects of race, ethnicity, socioeconomic status (SES), and disease severity on acute care utilization in patients with glomerular disease are unknown.Prospective cohort study.1,456 adults and 768 children with biopsy-proven glomerular disease enrolled in the Cure Glomerulonephropathy (CureGN) cohort.Race and ethnicity as a participant-reported social factor.Acute care utilization defined as hospitalizations or emergency department visits.Multivariable recurrent event proportional rate models were used to estimate associations between race and ethnicity and acute care utilization.Black or Hispanic participants had lower SES and more severe glomerular disease than White or Asian participants. Acute care utilization rates were 45.6, 29.5, 25.8, and 19.2 per 100 person-years in Black, Hispanic, White, and Asian adults, respectively, and 55.8, 42.5, 40.8, and 13.0, respectively, for children. Compared with the White race (reference group), Black race was significantly associated with acute care utilization in adults (rate ratio [RR], 1.76 [95% CI, 1.37-2.27]), although this finding was attenuated after multivariable adjustment (RR, 1.31 [95% CI, 1.03-1.68]). Black race was not significantly associated with acute care utilization in children; Asian race was significantly associated with lower acute care utilization in children (RR, 0.32 [95% CI 0.14-0.70]); no significant associations between Hispanic ethnicity and acute care utilization were identified.We used proxies for SES and lacked direct information on income, household unemployment, or disability.Significant differences in acute care utilization rates were observed across racial and ethnic groups in persons with prevalent glomerular disease, although many of these difference were explained by differences in SES and disease severity. Measures to combat socioeconomic disadvantage in Black patients and to more effectively prevent and treat glomerular disease are needed to reduce disparities in acute care utilization, improve patient wellbeing, and reduce health care costs.
Published: 2022

28. Vasculitic neuropathy associated with IgG4-related kidney disease: A case report and literature review

Author: Miriam Freimer, Zarife Sahenk, Isabelle Ayoub, Anjali A. Satoskar, and Benjamin Jiang
Subjects: medicine.medical_specialty, peripheral neuropathy, Anemia, Context (language use), Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Case Report, Malignancy, Kidney, Biopsy, parasitic diseases, medicine, Humans, IgG4-related disease, Peroxidase, medicine.diagnostic_test, business.industry, Peripheral Nervous System Diseases, General Medicine, Middle Aged, medicine.disease, Dermatology, Peripheral neuropathy, Nephrology, Female, Immunoglobulin G4-Related Disease, Differential diagnosis, Vasculitis, business, ANCA-associated vasculitis, Kidney disease
Abstract: IgG4-related disease is an immune-mediated systemic inflammatory condition characterized by tissue infiltration of IgG4-positive plasma cells and elevated serum IgG4 concentrations. Peripheral neuropathy is an atypical manifestation of this disease. We describe an unusual case of vasculitic neuropathy in a patient with IgG4-related kidney disease. A 55-year-old woman presented with right leg weakness progressing to bilateral leg weakness, pain and numbness of the legs, and impaired gait. She was previously evaluated for weight loss and anemia with a CT scan of the abdomen due to concern for malignancy. Abnormal enhancement of the kidneys was seen, and laboratory work-up and kidney biopsy were consistent with IgG4-related disease. Myeloperoxidase-antineutrophil cytoplasmic antibodies were also positive. In combination with the patient's asymmetric leg weakness and painful neuropathy, this raised concern for vasculitis. Sural nerve biopsy confirmed vasculitic neuropathy. Recent studies have demonstrated an overlap in the clinical characteristics of IgG4-related disease and the anti-neutrophil cytoplasmic antibody-associated vasculitides, which are known to cause vasculitic neuropathy. Clinicians should recognize this association, and IgG4-related disease should be considered in the differential diagnosis in patients with peripheral neuropathy in the right clinical context.
Published: 2021

29. What every nephrologist needs to know about hydroxychloroquine toxicity

Author: Sergey V. Brodsky, Stacy P. Ardoin, Isabelle Ayoub, Priyamvada Singh, and Lee A. Hebert
Subjects: Adult, Nephrology, medicine.medical_specialty, MEDLINE, Kidney, urologic and male genital diseases, Nephrologists, Retinal Diseases, Internal medicine, medicine, Humans, Lupus Erythematosus, Systemic, skin and connective tissue diseases, Intensive care medicine, Lupus erythematosus, business.industry, Hydroxychloroquine, General Medicine, medicine.disease, stomatognathic diseases, Antirheumatic Agents, Female, business, medicine.drug
Abstract: Hydroxychloroquine (HCQ) has become the rheumatologists's "Swiss army knife" when it comes to managing the rheumatologic manifestations of SLE and other auto-immune disorders. By contrast, nephrologists are much less comfortable in managing the multifaceted effect of HCQ. As a result, nephrologists are inclined to abdicate their responsibility for HCQ management, if this therapy was initiated by a rheumatologist. This report describes such a situation, which had devastating consequences for the patient. On this basis we suggest that this report is a story worth telling, and should encourage the nephrologist to be more involved in their patients' HCQ management.
Published: 2020

30. Management and treatment of glomerular diseases (part 2): conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference

Author: Brad H. Rovin, Dawn J. Caster, Daniel C. Cattran, Keisha L. Gibson, Jonathan J. Hogan, Marcus J. Moeller, Dario Roccatello, Michael Cheung, David C. Wheeler, Wolfgang C. Winkelmayer, Jürgen Floege, Sharon G. Adler, Charles E. Alpers, Isabelle Ayoub, Arvind Bagga, Sean J. Barbour, Jonathan Barratt, Daniel T.M. Chan, Anthony Chang, Jason Chon Jun Choo, H. Terence Cook, Rosanna Coppo, Fernando C. Fervenza, Agnes B. Fogo, Jonathan G. Fox, Richard J. Glassock, David Harris, Elisabeth M. Hodson, Elion Hoxha, Kunitoshi Iseki, J. Charles Jennette, Vivekanand Jha, David W. Johnson, Shinya Kaname, Ritsuko Katafuchi, A. Richard Kitching, Richard A. Lafayette, Philip K.T. Li, Adrian Liew, Jicheng Lv, Ana Malvar, Shoichi Maruyama, Juan Manuel Mejía-Vilet, Chi Chiu Mok, Patrick H. Nachman, Carla M. Nester, Eisei Noiri, Michelle M. O'Shaughnessy, Seza Özen, Samir M. Parikh, Hyeong-Cheon Park, Chen Au Peh, William F. Pendergraft, Matthew C. Pickering, Evangéline Pillebout, Jai Radhakrishnan, Manish Rathi, Pierre Ronco, William E. Smoyer, Sydney C.W. Tang, Vladimír Tesař, Joshua M. Thurman, Hernán Trimarchi, Marina Vivarelli, Giles D. Walters, Angela Yee-Moon Wang, Scott E. Wenderfer, Jack F.M. Wetzels, Baylor College of Medicine (BCM), Baylor University, Division of Nephrology, Rheinisch-Westfälische Technische Hochschule Aachen (RWTH), Department of Pediatrics, Division of Pediatric Nephrology and Genetics, All India Institute of Medical Sciences, University of British Columbia (UBC), Science of Turin Health Agency [Turin, Italy] (City of the Health), Regina Margherita University Children's Hospital [Turin, Italy], Mayo Clinic [Rochester], University College of London [London] (UCL), Molecular Otolaryngology and Renal Research Laboratories [Iowa City, IA, USA] (Carver College of Medicine), University of Iowa [Iowa City]-Carver College of Medicine, University of Iowa, Centre for Complement and Inflammation Research [London, UK] (Department of Medicine), Imperial College London, Service de rhumatologie, CHU Bordeaux [Bordeaux], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), University of Colorado [Denver], Department of Medicine, The University of Hong Kong (HKU), Department of Nephrology [Nijmegen, The Netherlands], and Radboud University Medical Centre [Nijmegen, The Netherlands]
Subjects: 0301 basic medicine, Nephrology, medicine.medical_specialty, membranoproliferative glomerulonephritis, Consensus Development Conferences as Topic, 030232 urology & nephrology, Lupus nephritis, Paraproteinemias, Context (language use), Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, anti-neutrophil cytoplasmic antibody–associated vasculitis, 03 medical and health sciences, 0302 clinical medicine, Glomerulonephritis, Risk Factors, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], Internal medicine, Membranoproliferative glomerulonephritis, medicine, Humans, Minimal change disease, C3 glomerulopathy, Genetic Testing, Intensive care medicine, ComputingMilieux_MISCELLANEOUS, KDIGO, lupus nephritis, business.industry, Podocytes, Nephrosis, Lipoid, monoclonal gammopathies of renal significance, Guideline, medicine.disease, focal and segmental glomerulosclerosis, 3. Good health, minimal change disease, 030104 developmental biology, Treatment Outcome, Practice Guidelines as Topic, Disease Progression, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], business, Biomarkers, Kidney disease, Glomerular Filtration Rate
Abstract: Contains fulltext : 203024.pdf (Publisher’s version ) (Open Access) In November 2017, the Kidney Disease: Improving Global Outcomes (KDIGO) initiative brought a diverse panel of experts in glomerular diseases together to discuss the 2012 KDIGO glomerulonephritis guideline in the context of new developments and insights that had occurred over the years since its publication. During this KDIGO Controversies Conference on Glomerular Diseases, the group examined data on disease pathogenesis, biomarkers, and treatments to identify areas of consensus and areas of controversy. This report summarizes the discussions on primary podocytopathies, lupus nephritis, anti-neutrophil cytoplasmic antibody-associated nephritis, complement-mediated kidney diseases, and monoclonal gammopathies of renal significance.
Published: 2019

31. Contributors

Author: Nancy Agmon-Levin, Graciela S. Alarcón, Olga Amengual, Stacy P. Ardoin, Swati Arora, Yemil Atisha-Fregoso, John P. Atkinson, Tatsuya Atsumi, Isabelle Ayoub, Maria-Louise Barilla-LaBarca, Bonnie L. Bermas, Sasha Bernatsky, George Bertsias, Tanmayee Bichile, Patrick Blanco, Miyuki Bohgaki, Gisela Bonsmann, Maria Orietta Borghi, Dimitrios T. Boumpas, Rebecka Bourn, Jill P. Buyon, Roberto Caricchio, Edward K.L. Chan, Christopher Chang, Manon Charrier, Cecilia Beatrice Chighizola, Ann E. Clarke, José C. Crispín, Bettina Cuneo, Thomas Dörner, Erika M. Damato, Alastair K.O. Denniston, Amy Devlin, Betty Diamond, T. Ernandez, Titilola Falasinnu, Ruth Fernandez-Ruiz, Brianna Fitzpatrick, Lindsy Forbess, Eleni A. Frangou, Marvin J. Fritzler, Shu Man Fu, Richard Furie, Felicia Gaskin, Dafna Gladman, Caroline Gordon, Amrie C. Grammer, Eric L. Greidinger, Teri M. Greiling, Shuhong Han, James E. Hansen, Sarfaraz A. Hasni, Fadi Hassan, Christian M. Hedrich, Keiju Hiromura, Diane Horowitz, Xin Huang, David Hunt, Peter M. Izmirly, Judith A. James, Wael N. Jarjour, Caroline A. Jefferies, Caroline Jefferies, Xiaoyue Jiang, Mariana J. Kaplan, Takayuki Katsuyama, Munther Khamashta, Kathryn M. Kingsmore, Takao Koike, Dwight H. Kono, Martin A. Kriegel, Annegret Kuhn, Vasileios C Kyttaris, Antonio La Cava, Alexandra Ladouceur, Robert G. Lahita, Aysche Landmann, Estibaliz Lazaro, Mara L. Lennard Richard, Andreia C. Lino, Peter E. Lipsky, M. Kathryn Liszewski, Mindy S. Lo, Qianjin Lu, Mary Mahieu, Susan Malkiel, Susan Manzi, Galina Marder, T.N. Mayadas, Pier Luigi Meroni, Joan T. Merrill, Chandra Mohan, Chi Chiu Mok, Vaishali R. Moulton, Philip I. Murray, Mohammad E. Naffaa, Masaomi Nangaku, Timothy Niewold, K. Okubo, Nancy J. Olsen, Trina Pal, Ziv Paz, Andras Perl, Guillermo J. Pons-Estel, Bo Qu, Anisur Rahman, Ziaur S.M. Raman, Rosalind Ramsey-Goldman, Westley H. Reeves, Christophe Richez, Florencia Rosetti, Brad H. Rovin, Robert L. Rubin, Stephanie Saeli, G. Saggu, Lisa R. Sammaritano, Minoru Satoh, Amr H. Sawalha, Amit Saxena, Savino Sciascia, Syahrul Sazliyana Shaharir, Amir Sharabi, Nan Shen, Robert H. Shmerling, Julia F. Simard, Vanja Sisirak, Samantha Slight-Webb, Isaac Ely Stillman, Sun-Sang J. Sung, Payal Thakkar, Argyrios N. Theofilopoulos, Donald E. Thomas, Jr, Hiromi Tissera, Zahi Touma, Betty P. Tsao, Manuel F. Ugarte-Gil, Murray B. Urowitz, Silvio Manfredo Vieira, Benjamin Wainwright, Daniel J. Wallace, Hongyang Wang, Haijing Wu, Soad Haj Yahia, C. Yung Yu, Zhenhuan Zhao, and Haoyang Zhuang
Published: 2021

32. The clinical evaluation of kidney disease in systemic lupus erythematosus

Author: Brad H. Rovin, Isabelle Ayoub, and Swati Arora
Published: 2021

33. Molecular profiling of kidney compartments from serial biopsies differentiate treatment responders from non-responders in lupus nephritis

Author: Samir V. Parikh, Ana Malvar, Huijuan Song, John Shapiro, Juan Manuel Mejia-Vilet, Isabelle Ayoub, Salem Almaani, Sethu Madhavan, Valeria Alberton, Celeste Besso, Bruno Lococo, Anjali Satoskar, Jianying Zhang, Lianbo Yu, Paolo Fadda, Michael Eadon, Dan Birmingham, Latha P. Ganesan, Wael Jarjour, and Brad H. Rovin
Subjects: Integrins, Nephrology, Biopsy, Humans, RNA, Complement C5a, Complement System Proteins, Interferons, Kidney, Lupus Nephritis, Biomarkers, Fibronectins
Abstract: The immune pathways that define treatment response and non-response in lupus nephritis (LN) are unknown. To characterize these intra-kidney pathways, transcriptomic analysis was done on protocol kidney biopsies obtained at flare (initial biopsy (Bx1)) and after treatment (second biopsy (Bx2)) in 58 patients with LN. Glomeruli and tubulointerstitial compartments were isolated using laser microdissection. RNA was extracted and analyzed by nanostring technology with transcript expression from clinically complete responders, partial responders and non-responders compared at Bx1 and Bx2 and to the healthy controls. Top transcripts that differentiate clinically complete responders from non-responders were validated at the protein level by confocal microscopy and urine ELISA. At Bx1, cluster analysis determined that glomerular integrin, neutrophil, chemokines/cytokines and tubulointerstitial chemokines, T cell and leukocyte adhesion genes were able to differentiate non-responders from clinically complete responders. At Bx2, glomerular monocyte, extracellular matrix, and interferon, and tubulointerstitial interferon, complement, and T cell transcripts differentiated non-responders from clinically complete responders. Protein analysis identified several protein products of overexpressed glomerular and tubulointerstitial transcripts at LN flare, recapitulating top transcript findings. Urine complement component 5a and fibronectin-1 protein levels reflected complement and fibronectin expression at flare and after treatment. Thus, transcript analysis of serial LN kidney biopsies demonstrated how gene expression in the kidney changes with clinically successful and unsuccessful therapy. Hence, these insights into the molecular landscape of response and non-response may help align LN management with the pathogenesis of kidney injury.
Published: 2020

34. Advances in ANCA-associated vasculitis and lupus nephritis

Author: Isabelle, Ayoub and Patrick H, Nachman
Subjects: Humans, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Lupus Nephritis
Published: 2020

35. The Use of Glucocorticoids in Lupus Nephritis: New Pathways for an Old Drug

Author: Isabelle Ayoub and Juan M. Mejia-Vilet
Subjects: Drug, media_common.quotation_subject, Lupus nephritis, Review, Bioinformatics, Disease activity, 03 medical and health sciences, 0302 clinical medicine, systemic lupus erythematosus, Prednisone, adverse effect, medicine, 030212 general & internal medicine, Adverse effect, media_common, lupus nephritis, 030203 arthritis & rheumatology, lcsh:R5-920, glucocorticoids, business.industry, Treatment development, General Medicine, medicine.disease, Treatment efficacy, methylpredisolone, prednisone, Medicine, lcsh:Medicine (General), business, hormones, hormone substitutes, and hormone antagonists, Glucocorticoid, steroids, medicine.drug
Abstract: Glucocorticoids therapy has greatly improved the outcome of lupus nephritis patients. Since their discovery, their adverse effects have counterbalanced their beneficial anti-inflammatory effects. Glucocorticoids exert their effects through both genomic and non-genomic pathways. Differential activation of these pathways is clinically relevant in terms of benefit and adverse effects. Ongoing aims in lupus nephritis treatment development focus on a better use of glucocorticoids combined with immunosuppressant drugs and biologics. Newer regimens aim to decrease the peak glucocorticoid dose, allow a rapid glucocorticoid tapering, and intend to control disease activity with a lower cumulative glucocorticoid exposure. In this review we discuss the mechanisms, adverse effects and recent strategies to limit glucocorticoid exposure without compromising treatment efficacy.
Published: 2020

36. Infection-related glomerulonephritis mimicking lupus nephritis

Author: Rasha Alawieh, Eshetu Obole, Anjali A. Satoskar, Lee A. Hebert, and Isabelle Ayoub
Subjects: Male, Pathology, medicine.medical_specialty, Lupus nephritis, Cardiac echo, Bacteremia, urologic and male genital diseases, Immunofluorescence, Kidney, Diagnosis, Differential, Streptococcus mutans, Glomerulonephritis, Streptococcal Infections, medicine, Humans, Aged, biology, medicine.diagnostic_test, business.industry, General Medicine, medicine.disease, Lupus Nephritis, Nephrology, Pauci-immune, biology.protein, Renal biopsy, Antibody, medicine.symptom, business
Abstract: The glomerulonephritis (GN) of granulomatosis polyangiitis is described as "pauci immune" because the glomeruli show little or no evidence of immune complex deposition by immunofluorescence or electron microscopy. Here we describe a severe crescentic GN in which the patient was myeloperoxidase-antineutrophil cytoplasmic antibodies (MPO-ANCA) positive, and on renal biopsy the glomeruli were pauci immune (there were only a few electron-dense deposits). However, by immunofluorescence the glomeruli showed "full-house" staining (the glomeruli stained positive for C1q, C3, IgG, IgA, and IgM). The latter staining pattern would be consistent with that seen in patients with lupus-like GN or with severe crescentic GN as a result of bacterial infection. So, should this patient receive high-dose immunosuppressive therapy and steroid therapy to treat presumed autoimmune GN, or should the patient receive intensive antibiotic therapy to treat a presumed underlying severe infection? This dilemma was soon resolved because the patient's blood culture returned positive for Streptococcus mutans and cardiac echo showed evidence of bacterial endocarditis. This report provides further detail regarding the patient's clinical issues.
Published: 2020

37. Multivesicular bodies mimicking SARS-CoV-2 in patients without COVID-19

Author: Edward Calomeni, Brad H. Rovin, Isabelle Ayoub, Tibor Nadasdy, Anjali A. Satoskar, and Sergey V. Brodsky
Subjects: Adult, Male, 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Pneumonia, Viral, Kidney, Article, Betacoronavirus, Pandemic, Medicine, Humans, In patient, Pandemics, Aged, Retrospective Studies, Aged, 80 and over, biology, business.industry, SARS-CoV-2, Multivesicular Bodies, COVID-19, Middle Aged, biology.organism_classification, Virology, Nephrology, Female, business, Coronavirus Infections
Published: 2020
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38. Frequency of Cytomegalovirus Seropositivity and Viremia in a Midwestern University Lupus Population

Author: Stacy P. Ardoin, Isabelle Ayoub, Holly Steigleman, Alexa Meara, Brad H. Rovin, Julliette Yedimenko, Brian LaMoreaux, Samir M. Parikh, and Wael N. Jarjour
Subjects: Adult, Male, Population, Congenital cytomegalovirus infection, MEDLINE, Cytomegalovirus, Viremia, Immunocompromised Host, Rheumatology, Seroepidemiologic Studies, Medicine, Humans, Lupus Erythematosus, Systemic, Serologic Tests, education, education.field_of_study, Systemic lupus erythematosus, business.industry, medicine.disease, United States, Cross-Sectional Studies, Immunology, Cytomegalovirus Infections, Female, business, Immunosuppressive Agents
Published: 2020

39. Spot Urine Protein/Creatinine Ratio Testing at a Large University Medical Center: Evidence for Overuse of This Low-Value Diagnostic Test

Author: Lee A. Hebert, Isabelle Ayoub, Daniel J. Birmingham, and JoAnna Williams
Subjects: lupus nephritis, medicine.medical_specialty, business.industry, Diagnostic test, lcsh:Diseases of the genitourinary system. Urology, lcsh:RC870-923, spot urine protein/creatinine ratio, Spot urine, Editorial, Nephrology, Clinical Research, Internal medicine, Protein/creatinine ratio, Medicine, University medical, business
Abstract: Introduction Cross-sectional studies document that the spot protein/creatinine ratio (PCR) is often an inaccurate estimate of proteinuria magnitude compared with the 24-hour PCR, which is the gold standard. However, the extent to which the inaccuracy of the spot PCR varies over time and between individuals has not previously been reported. We address these crucial questions using a unique database, an National Institutes of Health trial in which lupus nephritis (LN) patients (N = 103) provided spot PCR testing each month and 24-hour PCR testing every 3 months for up to 15 months after induction therapy. Methods A gold standard proteinuria trend line was constructed for each patient by joining the points that represented the serial 24-hour PCR values of the patient. The spot PCR values of the patient were then plotted in relationship to the 24-hour PCR trend line. Using our previous work, which estimated the 95% confidence intervals for the 24-hour PCR at specific levels, we determined in each patient whether the spot PCR values were “reliable,” “problematic,” or “unreliable.” The sequential spot PCR of the patients deviated widely and often from the 24-hour PCR trend line, to the extent that, if the spot PCR results were used in real time for clinical decision-making, it was likely management errors would occur. Results Spot PCRs were reliable in 41%, problematic in 24%, and unreliable in 35% of patients. Those with unreliable spot PCRs could not be predicted and were more likely to respond poorly to treatment. Conclusion The spot PCR should not be used for management of LN, and perhaps, other glomerulopathies.
Published: 2020

40. Limited Reliability of the Spot Urine Protein/Creatinine Ratio in the Longitudinal Evaluation of Patients With Lupus Nephritis

Author: David Wofsy, Daniel J. Birmingham, Brad H. Rovin, Ganesh Shidham, Isabelle Ayoub, Betty Diamond, Lee A. Hebert, and Paul L. Hebert
Subjects: 030203 arthritis & rheumatology, medicine.medical_specialty, Creatinine, Proteinuria, business.industry, 030232 urology & nephrology, Lupus nephritis, Gold standard (test), medicine.disease, Confidence interval, 3. Good health, Spot urine, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, chemistry, Nephrology, Internal medicine, medicine, Protein/creatinine ratio, medicine.symptom, business, Reliability (statistics)
Abstract: Introduction Cross-sectional studies document that the spot protein/creatinine ratio (PCR) is often an inaccurate estimate of proteinuria magnitude compared with the 24-hour PCR, which is the gold standard. However, the extent to which the inaccuracy of the spot PCR varies over time and between individuals has not previously been reported. We address these crucial questions using a unique database, an National Institutes of Health trial in which lupus nephritis (LN) patients (N = 103) provided spot PCR testing each month and 24-hour PCR testing every 3 months for up to 15 months after induction therapy. Methods A gold standard proteinuria trend line was constructed for each patient by joining the points that represented the serial 24-hour PCR values of the patient. The spot PCR values of the patient were then plotted in relationship to the 24-hour PCR trend line. Using our previous work, which estimated the 95% confidence intervals for the 24-hour PCR at specific levels, we determined in each patient whether the spot PCR values were “reliable,” “problematic,” or “unreliable.” The sequential spot PCR of the patients deviated widely and often from the 24-hour PCR trend line, to the extent that, if the spot PCR results were used in real time for clinical decision-making, it was likely management errors would occur. Results Spot PCRs were reliable in 41%, problematic in 24%, and unreliable in 35% of patients. Those with unreliable spot PCRs could not be predicted and were more likely to respond poorly to treatment. Conclusion The spot PCR should not be used for management of LN, and perhaps, other glomerulopathies.
Published: 2018

41. Abatacept efficacy in steroid-resistant minimal-change disease revealed by the speed of proteinuria reduction after the start of abatacept

Author: Brad H. Rovin, Lee A. Hebert, David Dado, Tibor Nadasdy, Samir M. Parikh, and Isabelle Ayoub
Subjects: medicine.medical_specialty, Nephrosis, 030232 urology & nephrology, Spontaneous remission, 030204 cardiovascular system & hematology, urologic and male genital diseases, Gastroenterology, Abatacept, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Humans, Minimal change disease, Proteinuria, Glomerulosclerosis, Focal Segmental, urogenital system, business.industry, Nephrosis, Lipoid, nutritional and metabolic diseases, Glomerulosclerosis, General Medicine, medicine.disease, Steroid resistant, eye diseases, female genital diseases and pregnancy complications, Nephrology, medicine.symptom, business, Nephrotic syndrome, Immunosuppressive Agents, medicine.drug
Abstract: A unique characteristic of the response of minimal-change disease (MCD) or focal and segmental glomerulosclerosis (FSGS) to steroid therapy is that the remission of proteinuria occurs quickly, for example, within 4 - 6 weeks of the onset of steroid therapy, even in those with severe nephrotic syndrome. Remission of proteinuria in MCD and FSGS can also occur spontaneously (not steroid induced). However, spontaneous remission usually proceeds over several months or longer. Recently, there have been several reports that abatacept can induce proteinuria remission in MCD and FSGS. These claims, however, are dubious because either the remission occurred slowly over several months of abatacept therapy, or remission occurred within a few weeks of abatacept therapy, but the patient was also receiving therapies that could have accounted for the remission of proteinuria. Our case is unique in that his severe steroid- and cyclosporine-resistant MCD remitted acutely while receiving abatacept, and there was no other plausible explanation for the acute remission of his MCD. .
Published: 2018

42. Patient Outcomes in Renal-Limited Antineutrophil Cytoplasmic Antibody Vasculitis With Inactive Histology

Author: Isabelle Ayoub, Christian Pagnoux, Jennifer Scott, Alan D. Salama, Min Chen, Duvuru Geetha, Mark A. Little, Frank B. Cortazar, Zdenka Hruskova, and Tessa K. Novick
Subjects: medicine.medical_specialty, medicine.medical_treatment, 030232 urology & nephrology, Renal function, lcsh:RC870-923, urologic and male genital diseases, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Clinical Research, renal limited vasculitis, Internal medicine, Biopsy, medicine, Dialysis, Anti-neutrophil cytoplasmic antibody, 030203 arthritis & rheumatology, Kidney, medicine.diagnostic_test, business.industry, Immunosuppression, lcsh:Diseases of the genitourinary system. Urology, medicine.disease, medicine.anatomical_structure, Nephrology, Cohort, ANCA-associated vasculitis, Vasculitis, business, glomerulonephritis
Abstract: Introduction: Little is known about the anticipated disease course for individuals who present with renal-limited antineutrophil cytoplasmic antibody (ANCA)−associated vasculitis but who lack inflammation on a kidney biopsy. The impact of immunosuppression on renal and overall survival is unknown. Methods: Patients were recruited from 2005 to 2016 from 8 centers worldwide (N = 16) for this descriptive study. All had positive ANCA, elevated serum creatinine with active urine sediment, histologic evidence of pauci-immune glomerulonephritis without active lesions, and had no evidence of extrarenal vasculitis. We describe the characteristics of this cohort and the differences in the clinical, histologic, and therapeutic parameters of those who developed primary outcomes of end-stage renal disease (ESRD) and vasculitis relapse. Results: The cohort was 63% Caucasian, and 75% were men, with a median age of 62 years. At entry, the mean ± SD estimated glomerular filtration rate (eGFR) was 24 ± 20 ml/min per 1.73 m2, and 5 patients required dialysis. Twelve patients received immunosuppressive therapy, 25% experienced disease relapse, and 38% developed ESRD. Patients who developed ESRD had lower baseline eGFRs (8 ± 5 ml/min per 1.73 m2 vs. 35 ± 18 ml/min per 1.73 m2; P = 0.001) and more often required dialysis at presentation (83% vs. 0%; P = 0.001). Patients who relapsed were less likely to receive immunosuppression (25% for the relapsed group vs. 92% for the nonrelapsed group; relative risk: 0.27, risk difference: 67%; P = 0.03). Conclusion: Among these patients, lower initial eGFR and dialysis dependence at presentation might increase the risk for ESRD. Immunosuppression did not affect renal outcomes in this sample of patients but was associated with a reduced risk for vasculitis relapse. More information is needed on factors that predict treatment response in this high-risk group. Keywords: ANCA-associated vasculitis, glomerulonephritis, renal limited vasculitis
Published: 2018

43. Screening for cognitive impairment in SLE using the Self-Administered Gerocognitive Exam

Author: Songzhu Zhao, Alexa Meara, Holly Steigelman, H Madhoun, Stacy P. Ardoin, Isabelle Ayoub, Brad H. Rovin, N Davidson, Lee A. Hebert, Samir M. Parikh, Wael N. Jarjour, and Guy Brock
Subjects: Adult, Male, Self-Assessment, Central nervous system, Affect (psychology), Severity of Illness Index, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, medicine, Humans, Cognitive Dysfunction, 030212 general & internal medicine, Cognitive impairment, Ohio, 030203 arthritis & rheumatology, Autoimmune disease, Lupus erythematosus, business.industry, Self, Lupus Vasculitis, Central Nervous System, fungi, Cognition, Hispanic or Latino, Middle Aged, medicine.disease, Logistic Models, medicine.anatomical_structure, Multivariate Analysis, Immunology, Income, Female, business
Abstract: Objective Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can affect the central nervous system in multiple ways, including causing cognitive dysfunction. Cognitive dysfunction is a common complaint of SLE patients yet diagnosis is challenging, time consuming, and costly. This study evaluated the Self-Administered Gerocognitive Exam (SAGE) as a screening test for cognitive impairment in a cohort of SLE patients. Methods A total of 118 SLE patients completed the SAGE. Providers completed the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborative Clinics Damage Index (SLICC-DI). SAGE scores were grouped into normal (>16) and abnormal (≤16) categories. Univariate and multivariate analyses were performed. Results Of the 118 participants, 21(18%) scored ≤16 on the SAGE instrument. In univariate analysis, race, ethnicity, household income, and SLICC-DI scores were associated with the SAGE ( p $50,000, 95% CI 2.45–57, p = 0.002). Conclusions In SLE patients, this study demonstrates an independent relationship between neurocognitive impairment (as measured by the SAGE) and higher lupus-related damage, as measured by the SLICC-DI, and lower household income. Abnormal SAGE scores were also associated with Hispanic ethnicity. A language barrier could explain this because the SAGE instrument was conducted in English only. The SAGE was feasible to measure in the clinic setting.
Published: 2018

44. Bacterial endocarditis manifesting as autoimmune pulmonary renal syndrome: ANCA-associated lung hemorrhage and pauci-immune crescentic glomerulonephritis

Author: Isabelle Ayoub, Anjali A. Satoskar, Samer Mohandes, and Lee A. Hebert
Subjects: Lung Diseases, Male, Pathology, medicine.medical_specialty, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Hemorrhage, Antibodies, Antineutrophil Cytoplasmic, Serology, Diagnosis, Differential, 03 medical and health sciences, Glomerulonephritis, 0302 clinical medicine, Pulmonary-renal syndrome, Biopsy, medicine, Humans, Endocarditis, 030203 arthritis & rheumatology, Kidney, medicine.diagnostic_test, business.industry, Endocarditis, Bacterial, General Medicine, Middle Aged, medicine.disease, medicine.anatomical_structure, Nephrology, 030220 oncology & carcinogenesis, Pauci-immune, Acute Disease, Etiology, medicine.symptom, business
Abstract: The etiology of pulmonary renal syndrome can be broadly divided into infectious and autoimmune (predominantly ANCA vasculitis). The importance of timely differentiating between them stems from the deleterious effects of their respective treatment if misdiagnosed. Serology and tissue evaluation by pathology are employed to aid in this, however, in rare cases, this can be difficult. We present a case of infectious endocarditis that presented with pulmonary renal syndrome but had positive ANCA serology and a pauci-immune glomerulonephritis picture on kidney biopsy that posed diagnostic difficulty. Factors most helpful in differentiating between the two conditions are highlighted as well as treatment options. .
Published: 2018

45. Advances in ANCA-associated vasculitis and lupus nephritis

Author: Patrick H. Nachman and Isabelle Ayoub
Subjects: 0301 basic medicine, business.industry, 030232 urology & nephrology, Lupus nephritis, Autoantibody, ANCA-Associated Vasculitis, medicine.disease, Clinical trial, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, immune system diseases, Nephrology, Immunology, medicine, skin and connective tissue diseases, business, Vasculitis, Glomerular diseases
Abstract: In 2020 a number of clinical trials have provided insights into therapeutic approaches for the treatment of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis and lupus nephritis. Moreover, mechanistic insights have potential to open new therapeutic strategies in the future.
Published: 2020

46. The Kidney Biopsy in Systemic Lupus Erythematosus: A View of the Past and a Vision of the Future

Author: Salem Almaani, Isabelle Ayoub, Brad H. Rovin, Clarissa A. Cassol, and Samir V. Parikh
Subjects: Pathology, medicine.medical_specialty, Biopsy, 030232 urology & nephrology, Lupus nephritis, 030204 cardiovascular system & hematology, Kidney, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Lupus Erythematosus, Systemic, Pathology, Molecular, medicine.diagnostic_test, Molecular pathology, business.industry, medicine.disease, Precision medicine, Prognosis, Lupus Nephritis, medicine.anatomical_structure, Nephrology, Treatment decision making, business, Kidney disease
Abstract: The kidney biopsy advanced our understanding of kidney disease in systemic lupus erythematosus. It allowed for better recognition and classification of lupus nephritis (LN). Several LN classifications have been devised in an effort to inform treatment decision and predict prognosis, and these are being further updated. In this review, we will examine the role of diagnostic as well as repeat kidney biopsy in the management of LN, including the potential role of molecular interrogation as a step forward beyond conventional histology to guide the discovery of novel biomarkers and a precision medicine approach to the management of LN.
Published: 2019

47. Commentary on the Current Guidelines for the Diagnosis of Lupus Nephritis Flare

Author: Brad H. Rovin, Lee A. Hebert, Daniel J. Birmingham, and Isabelle Ayoub
Subjects: 0301 basic medicine, medicine.medical_specialty, Lupus nephritis, Urine collection device, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Rheumatology, Internal medicine, medicine, Humans, Lupus Erythematosus, Systemic, Clinical significance, Pyuria, skin and connective tissue diseases, Prospective cohort study, Hematuria, 030203 arthritis & rheumatology, Creatinine, Proteinuria, business.industry, Glomerulonephritis, Symptom Flare Up, medicine.disease, Lupus Nephritis, 030104 developmental biology, chemistry, Practice Guidelines as Topic, medicine.symptom, business
Abstract: Lupus nephritis flare is a frequent complication in patients with systemic lupus erythematosus. Recognizing disease activity is crucial in lupus nephritis management. Proteinuria magnitude and urine sediment change are major clinical indicators of lupus nephritis activity. This work updates these insights in light of recent findings regarding proteinuria quantification and urine sediment analyses. Currently, BILAG and SLEDAI estimate proteinuria magnitude based on the protein/creatinine ratio of “spot” (single void collections) or “intended” 24-h urine collections without specifying the extent to which the collection approaches a 24-h collection. As discussed here, and based on our recently published work, these approaches often incur serious errors that can adversely affect SLE patient management. Also incorporated into this work is a new analysis of the clinical significance of urine sediment hematuria and pyuria changes with regard to recent-onset SLE glomerulonephritis (GN) flare. This analysis is based on a prospective study of urine sediment changes in the Ohio SLE Study, which was an NIH-sponsored prospective observational study of SLE GN patients with SLE flare of recent onset. We propose that BILAG and SLEDAI renal flare criteria can be made more rigorous by incorporating recently published insights into proteinuria quantification using the protein/creatinine ratio of an intended 24-h urine collection that is at least 50% complete based on its creatinine content. Also proposed are new insights into the interpretation of urine sediment hematuria and pyuria based on findings from the Ohio SLE Study.
Published: 2019

48. Management and treatment of glomerular diseases (part 1): conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference

Author: Jürgen Floege, Sean J. Barbour, Daniel C. Cattran, Jonathan J. Hogan, Patrick H. Nachman, Sydney C.W. Tang, Jack F.M. Wetzels, Michael Cheung, David C. Wheeler, Wolfgang C. Winkelmayer, Brad H. Rovin, Sharon G. Adler, Charles E. Alpers, Isabelle Ayoub, Arvind Bagga, Jonathan Barratt, Dawn J. Caster, Daniel T.M. Chan, Anthony Chang, Jason Chon Jun Choo, H. Terence Cook, Rosanna Coppo, Fernando C. Fervenza, Agnes B. Fogo, Jonathan G. Fox, Keisha L. Gibson, Richard J. Glassock, David Harris, Elisabeth M. Hodson, Elion Hoxha, Kunitoshi Iseki, J. Charles Jennette, Vivekanand Jha, David W. Johnson, Shinya Kaname, Ritsuko Katafuchi, A. Richard Kitching, Richard A. Lafayette, Philip K.T. Li, Adrian Liew, Jicheng Lv, Ana Malvar, Shoichi Maruyama, Juan Manuel Mejía-Vilet, Marcus J. Moeller, Chi Chiu Mok, Carla M. Nester, Eisei Noiri, Michelle M. O'Shaughnessy, Seza Özen, Samir M. Parikh, Hyeong-Cheon Park, Chen Au Peh, William F. Pendergraft, Matthew C. Pickering, Evangéline Pillebout, Jai Radhakrishnan, Manish Rathi, Dario Roccatello, Pierre Ronco, William E. Smoyer, Vladimír Tesař, Joshua M. Thurman, Hernán Trimarchi, Marina Vivarelli, Giles D. Walters, Angela Yee-Moon Wang, Scott E. Wenderfer, Division of Nephrology, Rheinisch-Westfälische Technische Hochschule Aachen (RWTH), University of British Columbia (UBC), Department of Nephrology [Nijmegen, The Netherlands], Radboud University Medical Centre [Nijmegen, The Netherlands], Baylor College of Medicine (BCM), Baylor University, Department of Pediatrics, Division of Pediatric Nephrology and Genetics, All India Institute of Medical Sciences, Science of Turin Health Agency [Turin, Italy] (City of the Health), Regina Margherita University Children's Hospital [Turin, Italy], Mayo Clinic [Rochester], University College of London [London] (UCL), Molecular Otolaryngology and Renal Research Laboratories [Iowa City, IA, USA] (Carver College of Medicine), University of Iowa [Iowa City]-Carver College of Medicine, University of Iowa, Centre for Complement and Inflammation Research [London, UK] (Department of Medicine), Imperial College London, Service de rhumatologie, CHU Bordeaux [Bordeaux], CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), University of Colorado [Denver], Department of Medicine, and The University of Hong Kong (HKU)
Subjects: 0301 basic medicine, medicine.medical_specialty, Biopsy, Consensus Development Conferences as Topic, Kidney Glomerulus, 030232 urology & nephrology, Glomerulonephritis, Membranous, Nephropathy, 03 medical and health sciences, 0302 clinical medicine, Membranous nephropathy, Risk Factors, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], medicine, Humans, Glomerular disease, Renal Insufficiency, Chronic, Intensive care medicine, Glomerular diseases, ComputingMilieux_MISCELLANEOUS, Proteinuria, business.industry, Nephrosis, Lipoid, Glomerulonephritis, IGA, Guideline, medicine.disease, 3. Good health, 030104 developmental biology, Treatment Outcome, Nephrology, Practice Guidelines as Topic, Disease Progression, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], medicine.symptom, business, Kidney disease, Glomerular Filtration Rate
Abstract: Contains fulltext : 202673.pdf (Publisher’s version ) (Open Access) The Kidney Disease: Improving Global Outcomes (KDIGO) initiative organized a Controversies Conference on glomerular diseases in November 2017. The conference focused on the 2012 KDIGO guideline with the aim of identifying new insights into nomenclature, pathogenesis, diagnostic work-up, and, in particular, therapy of glomerular diseases since the guideline's publication. It was the consensus of the group that most guideline recommendations, in particular those dealing with therapy, will need to be revisited by the guideline-updating Work Group. This report covers general management of glomerular disease, IgA nephropathy, and membranous nephropathy.
Published: 2019

49. Staphylococcus Infection–Associated GN – Spectrum of IgA Staining and Prevalence of ANCA in a Single-Center Cohort

Author: Sarah Suleiman, Lee A. Hebert, Brad H. Rovin, Edward Calomeni, Isabelle Ayoub, Jessica Hemminger, Gyongyi Nadasdy, Anjali A. Satoskar, Tibor Nadasdy, Samir M. Parikh, Cherri Bott, and Sergey V. Brodsky
Subjects: Adult, Male, Nephrology, Immunoglobulin A, medicine.medical_specialty, Pathology, Epidemiology, Biopsy, 030232 urology & nephrology, Fluorescent Antibody Technique, Kidney, Critical Care and Intensive Care Medicine, Antibodies, Antineutrophil Cytoplasmic, Serology, Diagnosis, Differential, Young Adult, 03 medical and health sciences, Glomerulonephritis, 0302 clinical medicine, Internal medicine, medicine, Humans, Endocarditis, 030212 general & internal medicine, Aged, Aged, 80 and over, Transplantation, biology, medicine.diagnostic_test, business.industry, Glomerulonephritis, IGA, Original Articles, Complement C3, Middle Aged, Staphylococcal Infections, medicine.disease, Staining, Immunoglobulin G, Immunology, biology.protein, Female, business, Nephritis
Abstract: Background and objectives Staphylococcus infection–associated GN (SAGN) is a well recognized disease entity, particularly because of the frequent IgA-dominant glomerular immunoglobulin staining on kidney biopsy. Biopsy features can resemble two other disease entities – primary IgA nephropathy and Henoch-Schonlein purpura nephritis – posing a diagnostic pitfall. This is clinically relevant because of the crucial difference in the therapeutic approach. The diagnosis of SAGN is further complicated by the variability in the degree of glomerular IgA (and C3) staining, the extent of electron dense immune-type deposits, and positive ANCA serology in some patients. Design, setting, participants, & measurements We performed a thorough histopathologic review of our single-center cohort of 78 culture-proven SAGN biopsies to assess the spectrum of IgA staining, prevalence of ANCA serology, prevalence of subepithelial “humps,” and other histologic features to distinguish from primary IgA nephropathy. Results Among the 78 SAGN biopsies, IgA staining was trace in 25%, mild in 19%, moderate in 44%, and strong in 12% of the cases. C3 was frequently moderate-to-strong but was trace in 14% of the biopsies. Concomitantly trace IgA, IgG, and C3 (pauci-immune pattern) was seen in 13%. Crescents were present in 35% of the SAGN biopsies. Out of 41 patients tested for ANCA, nine (22%) were positive, including patients with endocarditis and other infections. Subepithelial humps were identified in only 31% of the SAGN biopsies. Conclusions SAGN biopsies show marked variability in IgA immunofluorescence staining and low frequency of subepithelial humps compared with poststreptococcal GN. Occasional ANCA positivity is present in cases of SAGN, even in infections other than endocarditis. Therefore, biopsy diagnosis can be difficult particularly when clinical symptoms of infection are subtle. Both the pathologist and the nephrologist should be aware of these diagnostic pitfalls.
Published: 2016

50. Quiz Page February 2016

Author: Isabelle Ayoub, Brad H. Rovin, Anthony Alvarado, Samir V. Parikh, and Salem Almaani
Subjects: 030203 arthritis & rheumatology, medicine.medical_specialty, Pathology, business.industry, 030232 urology & nephrology, Acute kidney injury, Azathioprine, medicine.disease, 03 medical and health sciences, 0302 clinical medicine, Nephrology, Internal medicine, Medicine, business, Granulomatosis with polyangiitis, medicine.drug
Published: 2016

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