Pediatric Nephrology and Rheumatology Practice Patterns in Granulomatosis with Polyangiitis: A Midwest Pediatric Nephrology Consortium Study

الملخص EN

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.