The rheumatology team was consulted and the diagnosis of GPA was made according to EULAR/PRINTO/PRES criteria for childhood GPA based on sinus, pulmonary, and renal (focally crescentic, pauci-immune glomerulonephritis) involvement in addition to elevated inflammatory markers and a positive PR3. The gene discussed is PRTN3; the disease is granulomatosis with polyangiitis.