Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study

David T Selewski¹, Josephine M Ambruzs², Gerald B Appel³, Andrew S Bomback³, Raed Bou Matar⁴, Yi Cai⁵, Daniel C Cattran⁶, Aftab S Chishti⁷, Vivette D D'Agati⁸, Cynthia J D'Alessandri-Silva⁹, Rasheed A Gbadegesin¹⁰, Jonathan J Hogan¹¹, Sandra Iragorri¹², J Charles Jennette¹³, Bruce A Julian¹⁴, Myda Khalid¹⁵, Richard A Lafayette¹⁶, Helen Liapis², Francesca Lugani¹⁷, Sarah A Mansfield¹⁸, Sherene Mason⁹, Patrick H Nachman¹⁹, Cynthia C Nast²⁰, Carla M Nester^{21

22}, Damien G Noone²³, Jan Novak²⁴, Michelle M O'Shaughnessy¹⁶, Heather N Reich⁶, Michelle N Rheault²⁵, Dana V Rizk¹⁴, Manish K Saha²⁶, Neil S Sanghani²⁷, C John Sperati²⁸, Rajasree Sreedharan²⁹, Tarak Srivastava³⁰, Agnieszka Swiatecka-Urban³¹, Katherine Twombley³², Tetyana L Vasylyeva³³, Donald J Weaver³⁴, Hong Yin³⁵, Jarcy Zee¹⁸, Ronald J Falk²⁶, Ali G Gharavi³, Brenda W Gillespie³⁶, Debbie S Gipson¹, Larry A Greenbaum³⁵, Lawrence B Holzman³⁷, Matthias Kretzler^{38

39}, Bruce M Robinson¹⁸, William E Smoyer^{40

41}, Michael Flessner⁴², Lisa M Guay-Woodford⁴³, Krzysztof Kiryluk³; CureGN Consortium

Collaborators, Affiliations

Collaborators

CureGN Consortium:
Ali Gharavi, Wooin Ahn, Gerald B Appel, Rupali S Avasare, Revekka Babayev, Ibrahim Batal, Andrew S Bomback, 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, Fangming Lin, Francesca Lugani, Maddalena Marasa, Glen Markowitz, Sumit Mohan, Krzysztof Mucha, Thomas L Nickolas, Jai Radhakrishnan, Maya K Rao, Renu Regunathan-Shenk, 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, Michael C Braun, Aftab Chishti, Donna Claes, Carl Cramer, Keefe Davis, Elif Erkan, Daniel Feig, Michael Freundlich, Melisha Hanna, Guillermo Hidalgo, Amrish Jain, Myda Khalid, Mahmoud Kallash, Jerome C Lane, John Mahan, Nisha Mathews, Carla Nester, Cynthia Pan, Hiren Patel, Adelaide Revell, Rajasree Sreedharan, Julia Steinke, Scott E Wenderfer, Craig S Wong, Ronald Falk, William Cook, Vimal Derebail, Agnes Fogo, Adil Gasim, Todd Gehr, Raymond Harris, Jason Kidd, Louis-Philippe Laurin, Will Pendergraft, Vincent Pichette, Thomas Brian Powell, Matthew B Renfrow, Virginie Royal, Lawrence B Holzman, 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 A Hladunewich, Jonathan Hogan, J Ashley Jefferson, Kenar Jhaveri, Duncan B Johnstone, Frederick Kaskel, Amy Kogan, Jeffrey Kopp, 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, C John Sperati, Agnes Swiatecka-Urban, Howard Trachtman, Katherine R Tuttle, Joseph Weisstuch, Olga Zhdanova, Brenda Gillespie, Debbie S Gipson, Matthias Kretzler, Bruce M Robinson, Laura Barisoni, Sarah Mansfield, Laura Mariani, Cynthia C Nast, Matthew Wladkowski, Jarcy Zee, Lisa M Guay-Woodford

Affiliations

¹ Division of Nephrology, Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan, USA.
² Arkana Laboratories, Little Rock, Arkansas, USA.
³ Department of Medicine, Division of Nephrology, Columbia University College of Physicians and Surgeons, New York, New York, USA.
⁴ Center for Pediatric Nephrology, Cleveland Clinic, Cleveland, Ohio, USA.
⁵ Division of Nephrology, Helen DeVos Children's Hospital, Grand Rapids, Michigan, USA.
⁶ Division of Nephrology, Department of Medicine, University Health Network, University of Toronto, Toronto, Ontario, Canada.
⁷ Division of Nephrology, Hypertension and Renal Transplantation, University of Kentucky, Lexington, Kentucky, USA.
⁸ Department of Pathology & Cell Biology, Columbia University Medical Center, New York, New York, USA.
⁹ Section of Pediatric Nephrology, Connecticut Children's Medical Center, Hartford, Connecticut, USA.
¹⁰ Department of Pediatrics, Division of Nephrology, Duke University Medical Center, Durham, North Carolina, USA.
¹¹ Renal Division, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
¹² Division of Nephrology and Hypertension, Department of Pediatrics, Oregon Health & Science University, Portland, Oregon, USA.
¹³ Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA.
¹⁴ Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.
¹⁵ Division of Nephrology, Department of Pediatrics, JW Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana, USA.
¹⁶ Division of Nephrology and Hypertension, Stanford University, Stanford, California, USA.
¹⁷ Pediatric Nephrology, Giannina Gasline Institute, Genova, Italy.
¹⁸ Arbor Research Collaborative for Health, Ann Arbor, Michigan, USA.
¹⁹ Division of Renal Diseases and Hypertension, Department of Medicine, University of Minnesota, Minneapolis, MN, USA.
²⁰ Department of Pathology, Cedars-Sinai Medical Center, Los Angeles, California, USA.
²¹ Division of Pediatric Nephrology, Dialysis and Transplantation, Stead Family Department of Pediatrics, University of Iowa, Iowa City, Iowa, USA.
²² Molecular Otolaryngology and Renal Research Laboratory, University of Iowa, Iowa City, Iowa, USA.
²³ Division of Nephrology, Department of Pediatrics, The Hospital for Sick Children, University Health Network, University of Toronto, Toronto, Ontario, Canada.
²⁴ Department of Microbiology, University of Alabama at Birmingham, Birmingham, Alabama, USA.
²⁵ Division of Nephrology, Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, USA.
²⁶ Division of Nephrology, Department of Medicine, University of North Carolina, Chapel Hill, North Carolina, USA.
²⁷ Division of Nephrology and Hypertension, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
²⁸ Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
²⁹ Division of Nephrology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
³⁰ Children's Mercy Hospital and University of Missouri-Kansas City School of Medicine, Kansas City, Missouri, USA.
³¹ Division of Nephrology, Department of Pediatrics, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
³² Division of Pediatric Critical Care Medicine, Medical University of South Carolina, Charleston, South Carolina, USA.
³³ Department of Pediatrics, Division of Nephrology, Texas Tech University Health Sciences Center, Lubbock, Texas, USA.
³⁴ Division of Nephrology and Hypertension, Levine Children's Hospital at Carolinas Medical Center, Charlotte, North Carolina, USA.
³⁵ Division of Pediatric Nephrology, Emory University and Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
³⁶ Department of Biostatistics, University of Michigan, Ann Arbor, Michigan, USA.
³⁷ Department of Medicine, University of Pennsylvania Medical School, Philadelphia, Pennsylvania, USA.
³⁸ Department of Computational Medicine and Bioinformatics, University of Michigan School of Medicine, Ann Arbor, Michigan USA.
³⁹ Division of Nephrology, Department of Internal Medicine, University of Michigan School of Medicine, Ann Arbor, Michigan, USA.
⁴⁰ Center for Clinical and Translational Research, The Research Institute at Nationwide Children's Hospital, Columbus, Ohio, USA.
⁴¹ Department of Pediatrics, The Ohio State University, Columbus, Ohio, USA.
⁴² Division of Kidney, Urology, and Hematology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA.
⁴³ Center for Translational Science, Children's National Health System, Washington, DC, USA.

PMID: 30450464
PMCID: PMC6224619
DOI: 10.1016/j.ekir.2018.07.021

Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study

David T Selewski et al. Kidney Int Rep. 2018.

. 2018 Aug 3;3(6):1373-1384.

doi: 10.1016/j.ekir.2018.07.021. eCollection 2018 Nov.

Authors

Collaborators

CureGN Consortium:
Ali Gharavi, Wooin Ahn, Gerald B Appel, Rupali S Avasare, Revekka Babayev, Ibrahim Batal, Andrew S Bomback, 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, Fangming Lin, Francesca Lugani, Maddalena Marasa, Glen Markowitz, Sumit Mohan, Krzysztof Mucha, Thomas L Nickolas, Jai Radhakrishnan, Maya K Rao, Renu Regunathan-Shenk, 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, Michael C Braun, Aftab Chishti, Donna Claes, Carl Cramer, Keefe Davis, Elif Erkan, Daniel Feig, Michael Freundlich, Melisha Hanna, Guillermo Hidalgo, Amrish Jain, Myda Khalid, Mahmoud Kallash, Jerome C Lane, John Mahan, Nisha Mathews, Carla Nester, Cynthia Pan, Hiren Patel, Adelaide Revell, Rajasree Sreedharan, Julia Steinke, Scott E Wenderfer, Craig S Wong, Ronald Falk, William Cook, Vimal Derebail, Agnes Fogo, Adil Gasim, Todd Gehr, Raymond Harris, Jason Kidd, Louis-Philippe Laurin, Will Pendergraft, Vincent Pichette, Thomas Brian Powell, Matthew B Renfrow, Virginie Royal, Lawrence B Holzman, 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 A Hladunewich, Jonathan Hogan, J Ashley Jefferson, Kenar Jhaveri, Duncan B Johnstone, Frederick Kaskel, Amy Kogan, Jeffrey Kopp, 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, C John Sperati, Agnes Swiatecka-Urban, Howard Trachtman, Katherine R Tuttle, Joseph Weisstuch, Olga Zhdanova, Brenda Gillespie, Debbie S Gipson, Matthias Kretzler, Bruce M Robinson, Laura Barisoni, Sarah Mansfield, Laura Mariani, Cynthia C Nast, Matthew Wladkowski, Jarcy Zee, Lisa M Guay-Woodford

Affiliations

¹ Division of Nephrology, Department of Pediatrics and Communicable Diseases, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Michigan, USA.
² Arkana Laboratories, Little Rock, Arkansas, USA.
³ Department of Medicine, Division of Nephrology, Columbia University College of Physicians and Surgeons, New York, New York, USA.
⁴ Center for Pediatric Nephrology, Cleveland Clinic, Cleveland, Ohio, USA.
⁵ Division of Nephrology, Helen DeVos Children's Hospital, Grand Rapids, Michigan, USA.
⁶ Division of Nephrology, Department of Medicine, University Health Network, University of Toronto, Toronto, Ontario, Canada.
⁷ Division of Nephrology, Hypertension and Renal Transplantation, University of Kentucky, Lexington, Kentucky, USA.
⁸ Department of Pathology & Cell Biology, Columbia University Medical Center, New York, New York, USA.
⁹ Section of Pediatric Nephrology, Connecticut Children's Medical Center, Hartford, Connecticut, USA.
¹⁰ Department of Pediatrics, Division of Nephrology, Duke University Medical Center, Durham, North Carolina, USA.
¹¹ Renal Division, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
¹² Division of Nephrology and Hypertension, Department of Pediatrics, Oregon Health & Science University, Portland, Oregon, USA.
¹³ Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA.
¹⁴ Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.
¹⁵ Division of Nephrology, Department of Pediatrics, JW Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana, USA.
¹⁶ Division of Nephrology and Hypertension, Stanford University, Stanford, California, USA.
¹⁷ Pediatric Nephrology, Giannina Gasline Institute, Genova, Italy.
¹⁸ Arbor Research Collaborative for Health, Ann Arbor, Michigan, USA.
¹⁹ Division of Renal Diseases and Hypertension, Department of Medicine, University of Minnesota, Minneapolis, MN, USA.
²⁰ Department of Pathology, Cedars-Sinai Medical Center, Los Angeles, California, USA.
²¹ Division of Pediatric Nephrology, Dialysis and Transplantation, Stead Family Department of Pediatrics, University of Iowa, Iowa City, Iowa, USA.
²² Molecular Otolaryngology and Renal Research Laboratory, University of Iowa, Iowa City, Iowa, USA.
²³ Division of Nephrology, Department of Pediatrics, The Hospital for Sick Children, University Health Network, University of Toronto, Toronto, Ontario, Canada.
²⁴ Department of Microbiology, University of Alabama at Birmingham, Birmingham, Alabama, USA.
²⁵ Division of Nephrology, Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, USA.
²⁶ Division of Nephrology, Department of Medicine, University of North Carolina, Chapel Hill, North Carolina, USA.
²⁷ Division of Nephrology and Hypertension, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
²⁸ Division of Nephrology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
²⁹ Division of Nephrology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
³⁰ Children's Mercy Hospital and University of Missouri-Kansas City School of Medicine, Kansas City, Missouri, USA.
³¹ Division of Nephrology, Department of Pediatrics, Children's Hospital of Pittsburgh of University of Pittsburgh Medical Center, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
³² Division of Pediatric Critical Care Medicine, Medical University of South Carolina, Charleston, South Carolina, USA.
³³ Department of Pediatrics, Division of Nephrology, Texas Tech University Health Sciences Center, Lubbock, Texas, USA.
³⁴ Division of Nephrology and Hypertension, Levine Children's Hospital at Carolinas Medical Center, Charlotte, North Carolina, USA.
³⁵ Division of Pediatric Nephrology, Emory University and Children's Healthcare of Atlanta, Atlanta, Georgia, USA.
³⁶ Department of Biostatistics, University of Michigan, Ann Arbor, Michigan, USA.
³⁷ Department of Medicine, University of Pennsylvania Medical School, Philadelphia, Pennsylvania, USA.
³⁸ Department of Computational Medicine and Bioinformatics, University of Michigan School of Medicine, Ann Arbor, Michigan USA.
³⁹ Division of Nephrology, Department of Internal Medicine, University of Michigan School of Medicine, Ann Arbor, Michigan, USA.
⁴⁰ Center for Clinical and Translational Research, The Research Institute at Nationwide Children's Hospital, Columbus, Ohio, USA.
⁴¹ Department of Pediatrics, The Ohio State University, Columbus, Ohio, USA.
⁴² Division of Kidney, Urology, and Hematology, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA.
⁴³ Center for Translational Science, Children's National Health System, Washington, DC, USA.

PMID: 30450464
PMCID: PMC6224619
DOI: 10.1016/j.ekir.2018.07.021

Abstract

Introduction: The Cure Glomerulonephropathy Network (CureGN) is a 66-center longitudinal observational study of patients with biopsy-confirmed minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (IgAN), including IgA vasculitis (IgAV). This study describes the clinical characteristics and treatment patterns in the IgA cohort, including comparisons between IgAN versus IgAV and adult versus pediatric patients.

Methods: Patients with a diagnostic kidney biopsy within 5 years of screening were eligible to join CureGN. This is a descriptive analysis of clinical and treatment data collected at the time of enrollment.

Results: A total of 667 patients (506 IgAN, 161 IgAV) constitute the IgAN/IgAV cohort (382 adults, 285 children). At biopsy, those with IgAV were younger (13.0 years vs. 29.6 years, P < 0.001), more frequently white (89.7% vs. 78.9%, P = 0.003), had a higher estimated glomerular filtration rate (103.5 vs. 70.6 ml/min per 1.73 m², P < 0.001), and lower serum albumin (3.4 vs. 3.8 g/dl, P < 0.001) than those with IgAN. Adult and pediatric individuals with IgAV were more likely than those with IgAN to have been treated with immunosuppressive therapy at or prior to enrollment (79.5% vs. 54.0%, P < 0.001).

Conclusion: This report highlights clinical differences between IgAV and IgAN and between children and adults with these diagnoses. We identified differences in treatment with immunosuppressive therapies by disease type. This description of baseline characteristics will serve as a foundation for future CureGN studies.

Keywords: Henoch-Schönlein purpura (HSP); IgA nephropathy (IgAN); IgA vasculitis (IgAV); glomerulonephritis.

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Figures

**Figure 1**
Distribution of patient age at biopsy, by IgA nephropathy (IgAN) and IgA vasculitis (IgAV).

See this image and copyright information in PMC

References

1. McGrogan A., Franssen C.F., de Vries C.S. The incidence of primary glomerulonephritis worldwide: a systematic review of the literature. Nephrol Dial Transplant. 2011;26:414–430. - PubMed
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Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study

Collaborators

Affiliations

Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study

Authors

Collaborators

Affiliations

Abstract

Figures

References

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