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

2023; Elsevier BV; Volume: 81; Issue: 6 Linguagem: Inglês

10.1053/j.ajkd.2022.11.012

1523-6838

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 M. Kidd, Aftab S. Chishti, Kimberly Reidy, Amy K. Mottl, Debbie S. Gipson, Tarak Srivastava, Katherine Twombley, Wooin Ahn, Gerald B. Appel, Paul S. Appelbaum, Revekka Babayev, Andrew S. Bomback, Brenda Chan, Vivette D. D’Agati, Samitri Dogra, Hilda Fernández, Ali G. Gharavi, William H. Hines, S. Ali Husain, Namrata G. Jain, Krzysztof Kiryluk, Fangming Lin, Maddalena Marasà, Glen S. Markowitz, Hila Milo Rasouly, Sumit Mohan, Nicola Mongera, Jordan G. Nestor, Thomas L. Nickolas, Jai Radhakrishnan, Maya K. Rao, Simone Sanna‐Cherchi, Shayan Shirazian, Michael B. Stokes, Natalie Uy, Anthony M. Valeri, Natalie Vena, Bartosz Foroncewicz, Barbara Moszczuk, Agnieszka Perkowska‐Ptasińska, Gian Marco Ghiggeri, Francesca Lugani, Josephine M. Ambruzs, Helen Liapis, Rossana Baracco, Amrish Jain, Isa Ashoor, Diego Avilés, 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 J. Weaver, Jerome C. Lane, Carl H. Cramer, Cindy Pan, Neil J. Paloian, Rajasree Sreedharan, Corinna Bowers, Mary Dreher, John D. Mahan, Samantha Sharpe, William E. Smoyer, Amira Al‐Uzri, Sandra Iragorri, Myda Khalid, Craig W. Belsha, Joseph L. Alge, Michael Braun, Angela C. Gomez, Scott E. Wenderfer, Tetyana L. Vasylyeva, Daniel I. Feig, Gabriel Cara‐Fuentes, Melisha Hannah, Carla Nester, Jon B. Klein, Chryso Katsoufis, Wacharee Seeherunvong, Michelle N. Rheault, Craig S. Wong, Nisha Mathews, John Barcia, Agnieszka Swiatecka‐Urban, Sharon Bartosh, Vikas R. Dharnidharka, Joseph P. Gaut, Louis‐Philippe Laurin, Virginie Royal, Anand Achanti, Milos N. Budisavljevic, Sally Self, Cybele Ghossein, Yonatan Peleg, Shikha Wadhwani, Salem Almaani, Tibor Nádasdy, Samir, Parikh, Brad H. Rovin, Anthony Chang, Huma Fatima, Bruce A. Julian, Jan Novák, Matthew B. Renfrow, Dana V. Rizk, Vimal K. Derebail, Ronald J. Falk, Keisha L. Gibson, Dorey A. Glenn, Susan L. Hogan, Koyal Jain, J. Charles Jennette, Caroline J. Poulton, Manish K. Saha, Agnes B. Fogo, Neil Sanghani, Selvaraj Muthusamy, Jeffrey R. Schelling, Jean Hou, Kevin V. Lemley, Warren Mika, Pierre Russo, Michelle Denburg, Amy J. Kogon, Kevin Meyers, Madhura Pradhan, Raed Bou Matar, John O’Toole, John R. Sedor, Christine B. Sethna, Suzanne Vento, Mohamed G. Atta, Serena M. Bagnasco, Alicia M. Neu, Sharon G. Adler, Tiane Dai, Ram Dukkipati, Fernando C. Fervenza, Sanjeev Sethi, Frederick J. Kaskel, Kaye Brathwaite, Joseph Weisstuch, Ming‐Fang Wu, Olga Zhdanova, Jurgen Heymann, Meryl Waldman, Cheryl A. Winkler, Katherine R. Tuttle, Jill Krissberg, Richard A. Lafayette, Kamal Fahmeedah, Elizabeth Talley, Michelle Hladunewich, Carmen Avila‐Casado, Daniel C. Cattran, Reich Heather, Philip Boll, Yelena Drexler, Alessia Fornoni, Patrick Gipson, Jeffrey B. Hodgin, Andrea L. Oliverio, Jon Hogan, Lawrence B. Holzman, Matthew Palmer, Gaia Coppock, Blaise Abromovitz, Michael Mortiz, Charles E. Alpers, J. Ashley Jefferson, Elizabeth Brown, Kamal Sambandam, Bethany Roehm, Bruce Robinson, Cynthia C. Nast, Laura Barisoni, Matthias Kretzler, Laura Mariani, Lisa M. Guay‐Woodford,

Renal Transplantation Outcomes and Treatments

Rationale & Objective Adolescent- and adult-onset 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 Glomerulonephropathy Network (CureGN) and assessed predictors of rituximab response. Study Design Prospective, multicenter, observational study. Study Participants CureGN participants with proven MCD on biopsy. Exposure Age at disease onset, initiation of renin-angiotensin-aldosterone system (RAAS) blockade, and immunosuppression including rituximab during the study period. Outcome Relapse and remission, change in estimated glomerular filtration rate (eGFR), and kidney failure. Analytical Approach Remission and relapse probabilities were estimated using Kaplan-Meier curves and gap time recurrent event models. Linear regression models were used for the outcome of change in eGFR. Cox proportional hazards models were used to estimate the association between rituximab administration and remission. Results The study included 304 childhood- (≤12 years old), 49 adolescent- (13-17 years old), and 201 adult- (≥18 years) onset participants with 2.7-3.2 years of follow-up after enrollment. Children had a longer time to biopsy (238 vs 23 and 36 days in adolescent- and adult-onset participants, respectively; P < 0.001) and were more likely to have received therapy before biopsy. Children were more likely to be treated with immunosuppression but not RAAS blockade. The rate of relapse was higher in childhood- versus adult-onset participants (HR, 1.69 [95% 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]). In all groups eGFR loss was minimal. Children were more likely to remit after rituximab than those with adolescent- or adult-onset disease (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). Limitations CureGN was limited to biopsy-proven disease. Comparisons of childhood to nonchildhood cases of MCD may be subject to selection bias, given that childhood cases who undergo a biopsy may be limited to patients who are least responsive to initial therapy. Conclusions Among patients with MCD who underwent kidney biopsy, there were differences in the course (relapse and remission) of childhood-onset compared with adolescent- and adult-onset disease, as well as rituximab response. Plain-Language Summary Minimal change disease is a biopsy diagnosis for nephrotic syndrome. It is diagnosed in childhood, adolescence, or adulthood. Patients and clinicians often have questions about what to expect in the disease course and how to plan therapies. We analyzed a group of patients followed longitudinally as part of the Cure Glomerulonephropathy Network (CureGN) and describe the differences in disease (relapse and remission) based on the age of onset. We also analyzed rituximab response. We found that those with childhood-onset disease had a higher rate of relapse but also have a higher probability of reaching remission when compared with adolescent- or adult-onset disease. Children and all steroid-responsive patients are more likely to achieve remission after rituximab. Adolescent- and adult-onset 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 Glomerulonephropathy 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 renin-angiotensin-aldosterone system (RAAS) blockade, and immunosuppression including rituximab during the study period. Relapse and remission, change in estimated glomerular filtration rate (eGFR), and kidney failure. Remission and relapse probabilities were estimated using Kaplan-Meier curves and gap time recurrent event models. Linear regression models were used for the outcome of change in eGFR. Cox proportional hazards models were used to estimate the association between rituximab administration and remission. The study included 304 childhood- (≤12 years old), 49 adolescent- (13-17 years old), and 201 adult- (≥18 years) onset participants with 2.7-3.2 years of follow-up after enrollment. Children had a longer time to biopsy (238 vs 23 and 36 days in adolescent- and adult-onset participants, respectively; P < 0.001) and were more likely to have received therapy before biopsy. Children were more likely to be treated with immunosuppression but not RAAS blockade. The rate of relapse was higher in childhood- versus adult-onset participants (HR, 1.69 [95% 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]). In all groups eGFR loss was minimal. Children were more likely to remit after rituximab than those with adolescent- or adult-onset disease (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 nonchildhood cases of MCD may be subject to selection bias, given that childhood cases who undergo a biopsy may be limited to 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 with adolescent- and adult-onset disease, as well as rituximab response.