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