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

CureGN Consortium

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8 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1373-1384
Number of pages12
JournalKidney International Reports
Volume3
Issue number6
DOIs
StatePublished - Nov 1 2018

Bibliographical note

Funding Information:
GBA has consulting fees or paid advisory boards for Alexion, Mallincrodt, Bristol-Meyers Squibb, Pfizer, Takeda, Genentech, Merck, and Sanofi, has received lecture fees from Takeda, Genentech, and Sanofi, and receives grant support from Bristol-Myers Squibb, Genentech, Regulus, Achillion and travel support from all consultations and grants. DCC has consulting fees or paid advisory boards with Mallincrodt and Nefigan for the known future and receives grant support from NIDDK/Neptune. VDD’A receives grant support from the National Institutes of Health (NIH)/NIDDK. CJDA-S received consulting fees/paid advisory board for Advicenne, receives grant support from CureGN, and has served as an expert witness in 2017 for a 1-day trial. JJH received consulting fees or paid advisory boards from Variant, Dimerix, GlaxoSmithKline, and Aurinia, and in the known future will receive them for Variant and Dimerix. BAJ has received consulting fees from Visterra, has equity ownership/stock options in Reliant Glycosciences, LLC, has grant support under negotiation from Retrophin and Shire, and has a US patent assigned to University of Alabama at Birmingham Research foundation. RAL receives consulting fees/paid advisory boards from Mallinckrodt, Inc., Rigel, Inc., and Genentech, Inc., and receives grant support for the MENTOR study, Protocol number NCT01180036 . HL has received consulting fees or paid advisory boards for Alexion and Michael and Wells Law Firm and receives grant support from the NIH. SM receives grant support from the Childhood Arthritis and Rheumatology Research Alliance. PHN receives grant support from NIDDK/Rare Disease Clinical Research Network. CMN has consulting fees/paid advisory boards from Achillion and receives grant support from Retrophine-Site PI on Duet. JN has received consulting fees/paid advisory boards from Visterra, has equity ownership/stock options in Reliant Glycosciences LLC, has grant support under negotiation with Retrophin and Shire, and has a US patent assigned to University of Alabama at Birmingham Research Foundation. MNR receives grant support from Reata, Retrophin, Regulus, and Novartis, and is negotiating with Roche. DVR has equity ownership/stock options in Reliant Glycosciences LLC, receives grant support from Reata Pharmaceuticals, Fast Biomedical, and AbbVie Inc. and has grant support under negotiation with Retrophin, Inc. and Calliditas. TS receives grant support from the NIH, Retrophin, Bristol-Myers Squibb, and Mallinckcroft. KT receives grant support from NIDDK, Takeda/Arbor, Alexion, Novartis, and Kaneka. DJW received lecture fees from Alexion Pharmaceuticals. BWG receives grant support for CureGN and Neptune. DSG has consulting agreements between the University of Michigan and the following entities: Janssen, Dimerix, and Bristol-Myers Squibb, and receives grant support from the NIH, the Centers for Disease Control and Prevention (CDC), the Patient-Centered Outcomes Research Institute (PCORI), Bristol-Myers Squibb, NephCure Kidney International, Variant, Inc., Complexa, Inc., Retrophin, Inc., Carolinas Medical Foundation, and the American Heart Association (total funding includes full project budgets [direct, indirect, and subcontracts to external sites]), and has funding under negotiation with Goldfinch Biopharma. LAG receives grant support from Bristol-Myers Squibb. LBH receives consulting fees or paid advisory boards from Bristol-Myers Squibb and in the known future with Bristol-Myers Squibb and receives grant support from the NIH. MK receives grant support from the NIH, University of Michigan, and Goldfinch Biopharma, and is in negotiation with Boehringer-Ingelheim. BMR is Principal Investigator for the Dialysis Outcomes and Practice Patterns Study (DOPPS) Program, which is supported by Amgen, Kyowa Hakko Kirin, AbbVie, Sanofi Renal, Baxter Healthcare, and Vifor Fresenius Medical Care Renal Pharma. Support for specific projects and countries is provided by Keryx Biopharmaceuticals, Merck Sharp & Dohme, Proteon Therapeutics, Relypsa, F. Hoffmann-LaRoche Benevolence Health Center (BHC) Medical Janssen Takeda, the Kidney Foundation of Canada Hexal DGfN Shire, and the WiNe Institute; for Peritoneal Dialysis Outcomes and Practice Patterns Study (PDOPPS) the Japanese Society for Peritoneal Dialysis; and NIH NIDDK funding for United States Renal Data System (USRDS) and CureGN. WES receives consulting fees/paid advisory boards from Pfizer External Pediatric Committee and receives grant support from NIDDK CureGN Ancillary R01 and the NIDDK CureGN Grant. LMG-W receives grant support from the NIH (National Center for Advancing Translational Sciences [NCATS], NIDDK, and National Institute of Child Health and Human Development [NICHD]). KK receives grant support from the NIDDK and IgA Foundation of America. All the other authors declared no competing interests.

Funding Information:
Funding for the CureGN consortium is provided by UM1DK100845 , UM1DK100846 , UM1DK100876 , UM1DK100866 , and UM1DK100867 from the NIDDK . Patient recruitment is supported by NephCure Kidney International . Jennifer McCready-Maynes, an employee of Arbor Research Collaborative for Health, provided editorial assistance.

Publisher Copyright:
© 2018 International Society of Nephrology

Keywords

  • Henoch-Schönlein purpura (HSP)
  • IgA nephropathy (IgAN)
  • IgA vasculitis (IgAV)
  • glomerulonephritis

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