Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

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; CureGN Consortium

doi:10.1016/j.ekir.2020.03.017

Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

Kidney Int Rep. 2020 Mar 20;5(6):860-871. doi: 10.1016/j.ekir.2020.03.017. eCollection 2020 Jun.

Authors

Elisa Delbarba^{1

2}, Maddalena Marasa¹, Pietro A Canetta¹, Stacy E Piva¹, Debanjana Chatterjee¹, Byum Hee Kil¹, Xueru Mu¹, Keisha L Gibson³, Michelle A Hladunewich⁴, Jonathan J Hogan⁵, Bruce A Julian⁶, Jason M Kidd⁷, Louis-Philippe Laurin⁸, Patrick H Nachman⁹, Michelle N Rheault¹⁰, Dana V Rizk⁶, Neil S Sanghani¹¹, Howard Trachtman¹², Scott E Wenderfer¹³, Ali G Gharavi¹, Andrew S Bomback¹; CureGN Consortium

Collaborators

CureGN Consortium:
Wooin Ahn, Gerald B Appel, Revekka Babayev, Ibrahim Batal, Andrew S Bomback, Eric Brown, Eric S Campenot, Pietro Canetta, Brenda Chan, Debanjana Chatterjee, Vivette D D'Agati, Elisa Delbarba, Hilda Fernandez, Bartosz Foroncewicz, Ali G Gharavi, Gian Marco Ghiggeri, William H Hines, Namrata G Jain, Byum Hee Kil, Krzysztof Kiryluk, Wai L Lau, Fangming Lin, Francesca Lugani, Maddalena Marasa, Glen Markowitz, Sumit Mohan, Xueru Mu, 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, Michelle N Rheault, Cynthia Silva, Rajasree Sreedharan, Tarak Srivastava, Julia Steinke, Katherine Twombley, Scott E Wenderfer, 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, Louis-Philippe Laurin, 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, Howard Trachtman, 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, Lisa M Guay-Woodford

Affiliations

¹ Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, New York, USA.
² Division of Nephrology, Spedali Civili and University of Brescia, Brescia, Italy.
³ Division of Nephrology, Department of Pediatrics, North Carolina Children's Hospital, Chapel Hill, North Carolina, USA.
⁴ Division of Nephrology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada.
⁵ Renal-Electrolyte and Hypertension Division, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
⁶ Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.
⁷ Division of Nephrology, Virginia Commonwealth University, Richmond, Virginia, USA.
⁸ Division of Nephrology, Maisonneuve-Rosemont Hospital, Department of Medicine, University of Montreal, Montreal, Quebec, Canada.
⁹ Department of Medicine, Division of Renal Diseases and Hypertension, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.
¹⁰ Division of Nephrology, Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, USA.
¹¹ Division of Nephrology and Hypertension, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
¹² Division of Nephrology, Department of Medicine and Pediatrics, New York University Langone Health and New York University School of Medicine, New York, New York, USA.
¹³ Renal Section, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA.

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 <5 years before enrollment drawn from the Cure Glomerulonephropathy Network (CureGN cohort, n = 1182; CUMC-CureGN cohort, n = 362). Disease activity was defined by (i) Kidney Disease: Improving Global Outcomes-recommended threshold criteria for initiation of immunosuppression in primary glomerulonephropathy (GN) and (ii) CureGN's Disease Activity Working Group definitions for activity.

Results: No significant differences were detected among the 3 cohorts in terms of age, sex, serum creatinine, and urinary protein-to-creatinine ratio. For each GN subtype, disease activity in the OLD cohort was comparable with disease activity in the entire CureGN and the CUMC-CureGN cohort. When limiting our comparisons to disease activity in incident CUMC-CureGN patients (first diagnostic biopsy within 6 months of enrollment), OLD patients demonstrated similar activity rates as incident patients.

Conclusion: Disease activity did not differ among patients with shorter versus longer duration of disease. Such survivor patients, with long-term but persistent disease, are potentially highly informative for understanding the clinical course and pathogenesis of GN and may help identify factors mediating more chronic subtypes of disease.

Keywords: IgA nephropathy; focal segmental glomerulosclerosis; glomerular disease; glomerulonephropathy; membranous nephropathy; minimal change disease.

Abstract

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