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Kidney Int Rep. 2018 Aug 3;3(6):1373-1384. doi: 10.1016/j.ekir.2018.07.021. eCollection 2018 Nov.

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

Collaborators (136)

Gharavi A, Ahn W, Appel GB, Avasare RS, Babayev R, Batal I, Bomback AS, Brown E, Campenot ES, Canetta P, Chan B, D'Agati VD, Fernandez H, Foroncewicz B, Ghiggeri GM, Hines WH, Jain NG, Kiryluk K, Lin F, Lugani F, Marasa M, Markowitz G, Mohan S, Mucha K, Nickolas TL, Radhakrishnan J, Rao MK, Regunathan-Shenk R, Sanna-Cherchi S, Santoriello D, Stokes MB, Yu N, Valeri AM, Zviti R, Greenbaum LA, Smoyer WE, Al-Uzri A, Ashoor I, Aviles D, Baracco R, Barcia J, Bartosh S, Belsha C, Braun MC, Chishti A, Claes D, Cramer C, Davis K, Erkan E, Feig D, Freundlich M, Hanna M, Hidalgo G, Jain A, Khalid M, Kallash M, Lane JC, Mahan J, Mathews N, Nester C, Pan C, Patel H, Revell A, Sreedharan R, Steinke J, Wenderfer SE, Wong CS, Falk R, Cook W, Derebail V, Fogo A, Gasim A, Gehr T, Harris R, Kidd J, Laurin LP, Pendergraft W, Pichette V, Powell TB, Renfrow MB, Royal V, Holzman LB, Adler S, Alpers C, Matar RB, Brown E, Cattran D, Choi M, Dell KM, Dukkipati R, Fervenza FC, Fornoni A, Gadegbeku C, Gipson P, Hasely L, Hingorani S, Hladunewich MA, Hogan J, Jefferson JA, Jhaveri K, Johnstone DB, Kaskel F, Kogan A, Kopp J, Lemley KV, Dieguez LM, Meyers K, Neu A, O'Shaughnessy MM, O'Toole JF, Parekh R, Reich H, Reidy K, Rondon H, Sambandam KK, Sedor JR, Selewski DT, Sethna CB, Schelling J, Sperati CJ, Swiatecka-Urban A, Trachtman H, Tuttle KR, Weisstuch J, Zhdanova O, Gillespie B, Gipson DS, Kretzler M, Robinson BM, Barisoni L, Mansfield S, Mariani L, Nast CC, Wladkowski M, Zee J, Guay-Woodford LM.

Author information

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.

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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