Proteinase 3

C-ANCA (90%)

P-ANCA (2%)

WG, microscopic polyarteritis, Churg–Strauss (30%)

Myeloperoxidase

P-ANCA (70%)

P-ANCA (5%)

WG, microscopic polyarteritis, Churg–Strauss (60%), GBM (30%)

Azuocidin (CAP37)

P-ANCA

Uncertain

β-glucuronidase

P-ANCA

Uncertain

Bactericidal permeability-increasing protein (BPI)

C-ANCA (4%) atypical ‘flat’

ANCA

Cystic fibrosis (50%)

Cathepsin G

P-ANCA (5%)

X-ANCA

Sclerosing cholangitis, ulcerative colitis

Elastase

P-ANCA (8%)

X-ANCA

SLE (neuro-SLE), hydralazine-induced SLE, RhA, ulcerative colitis

Lactoferrin

P-ANCA (10%)

X-ANCA

Inflammatory bowel disease, PBC, AIH, SLE, RhA

Lysozyme

C-ANCA

P-ANCA

Uncertain