The higher frequency of positive ANCA serology positivity (with proteinase-3 specificity/cANCA), higher frequency of purpuric skin rash, active glomerular crescents and necrotizing lesions on the kidney biopsy, and lack of positive blood cultures due to the fastidious nature of these Gram-negative bacilli increases the chances of misdiagnosis as ANCA-associated GN. This evidence concerns the gene PRTN3 and ganglioneuroma.