Pediatric Nephrology and Rheumatology Practice Patterns in Granulomatosis with Polyangiitis

A Midwest Pediatric Nephrology Consortium Study

Cristin D.W. Kaspar, Keia Sanderson, Seza Ozen, Priya Verghese, Megan Lo, Timothy E. Bunchman, Scott E. Wenderfer, Jason Kidd

Research output: Contribution to journalArticle

Abstract

Objective. To assess practice pattern similarities and differences amongst pediatric rheumatologists and nephrologists in the management of pediatric Granulomatosis with Polyangiitis (GPA). Methods. A voluntary survey was distributed to the Midwest Pediatric Nephrology Consortium Group (MWPNC) and an international pediatric rheumatology email listserv in 2016-2017. Data were collected on general practice characteristics and preferences for induction management under three clinical scenarios (A-C): newly diagnosed GPA with glomerulonephritis, GPA with rapidly progressive glomerulonephritis, and GPA with pulmonary hemorrhage. In addition, individual preferences for GPA maintenance medications, disease monitoring, and management of GPA with end-stage renal disease were ascertained. Results. There was a 68% response rate from the MWPNC membership and equal numbers of rheumatology respondents. Survey results revealed Rituximab plus Cyclophosphamide is a more common induction choice for rheumatologists than nephrologists in induction Scenarios A and B, whereas Cyclophosphamide is more commonly chosen by nephrologists in Scenario A. Plasmapheresis rates increased for Scenarios A, B, and C for both specialties, but were overall low. There was no clear consensus on the duration of maintenance therapy nor diagnostic work-up. Rheumatologists more frequently chose Rituximab for maintenance and induction compared to nephrologists. There was also a higher than expected proportion of Mycophenolate Mofetil use for both specialties. Conclusion. This survey has revealed important differences in the way that rheumatologists and nephrologists manage this disease. It highlights the need for well-designed clinical trials in pediatric GPA patients and reveals that both specialties must be represented during consensus-building and clinical trial design efforts.

Original languageEnglish (US)
Article number8413096
JournalInternational Journal of Nephrology
Volume2018
DOIs
StatePublished - Jan 1 2018

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Granulomatosis with Polyangiitis
Nephrology
Rheumatology
Pediatrics
Glomerulonephritis
Cyclophosphamide
Maintenance
Clinical Trials
Mycophenolic Acid
Plasmapheresis
Disease Management
General Practice
Chronic Kidney Failure
Nephrologists
Hemorrhage
Lung
Surveys and Questionnaires
Rheumatologists

PubMed: MeSH publication types

  • Journal Article

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Pediatric Nephrology and Rheumatology Practice Patterns in Granulomatosis with Polyangiitis : A Midwest Pediatric Nephrology Consortium Study. / Kaspar, Cristin D.W.; Sanderson, Keia; Ozen, Seza; Verghese, Priya; Lo, Megan; Bunchman, Timothy E.; Wenderfer, Scott E.; Kidd, Jason.

In: International Journal of Nephrology, Vol. 2018, 8413096, 01.01.2018.

Research output: Contribution to journalArticle

Kaspar, Cristin D.W. ; Sanderson, Keia ; Ozen, Seza ; Verghese, Priya ; Lo, Megan ; Bunchman, Timothy E. ; Wenderfer, Scott E. ; Kidd, Jason. / Pediatric Nephrology and Rheumatology Practice Patterns in Granulomatosis with Polyangiitis : A Midwest Pediatric Nephrology Consortium Study. In: International Journal of Nephrology. 2018 ; Vol. 2018.
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abstract = "Objective. To assess practice pattern similarities and differences amongst pediatric rheumatologists and nephrologists in the management of pediatric Granulomatosis with Polyangiitis (GPA). Methods. A voluntary survey was distributed to the Midwest Pediatric Nephrology Consortium Group (MWPNC) and an international pediatric rheumatology email listserv in 2016-2017. Data were collected on general practice characteristics and preferences for induction management under three clinical scenarios (A-C): newly diagnosed GPA with glomerulonephritis, GPA with rapidly progressive glomerulonephritis, and GPA with pulmonary hemorrhage. In addition, individual preferences for GPA maintenance medications, disease monitoring, and management of GPA with end-stage renal disease were ascertained. Results. There was a 68{\%} response rate from the MWPNC membership and equal numbers of rheumatology respondents. Survey results revealed Rituximab plus Cyclophosphamide is a more common induction choice for rheumatologists than nephrologists in induction Scenarios A and B, whereas Cyclophosphamide is more commonly chosen by nephrologists in Scenario A. Plasmapheresis rates increased for Scenarios A, B, and C for both specialties, but were overall low. There was no clear consensus on the duration of maintenance therapy nor diagnostic work-up. Rheumatologists more frequently chose Rituximab for maintenance and induction compared to nephrologists. There was also a higher than expected proportion of Mycophenolate Mofetil use for both specialties. Conclusion. This survey has revealed important differences in the way that rheumatologists and nephrologists manage this disease. It highlights the need for well-designed clinical trials in pediatric GPA patients and reveals that both specialties must be represented during consensus-building and clinical trial design efforts.",
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