Table 1.

The role of ANCA as biomarker for diagnosis and in the context of treatment response in AAV patients

Study	PR-3 AAV	MPO-AAV
Lyons et al. [8] (n = 1233/1454)	HLA-DP, SERPINA1, PRTN3	HLA-DQ
Lionaki et al. [9] (n = 502)	Higher relapse risk	Lower relapse risk
Cordova-Sanchez et al. [68] (n = 62)	Higher relapse risk	Lower relapse risk
Puechal et al. [69] (n = 59)	Higher relapse risk	Lower relapse risk
Schirmer et al. [70] (n = 315)	More than two organs involvement, more need for aggressive treatment (CYC/RTX), higher relapse risk	More limited disease (more prevalence of SG), less need for aggressive treatment (CYC/RTX), lower relapse risk
Solans-Laqué et al. [71] (n = 450)	More upper airway involvement, higher relapse risk, higher survival	More renal involvement and AH, lower relapse risk, lower survival
Chang et al. [72] (n = 455)	Milder renal involvement, higher relapse risk, more constitutional symptoms	More severe renal involvement, lower relapse risk, fewer constitutional symptoms
Unizony et al. [73] (n = 197) (NCT 00104299 post-results)	Better remission induction and maintenance with RTX than CYC/AZA	Similar remission induction and maintenance with RTX vs. CYC/AZA
Miloslavsky et al. [74] (n = 197) (NCT 00104299)	More uncontrolled disease, higher relapse risk, better remission with RTX than CYC/AZA	Less uncontrolled disease, lower relapse risk, similar remission with RTX vs. CYC/AZA
	Neither ANCA titers nor B cell counts predicted disease flare
Berti et al. [75] (n = 58)	Higher survival	Lower survival
Morgan et al. [76] (n = 252) (NCT 00430105)	Higher relapse risk	Lower relapse risk
	Becoming ANCA negative at the time of switching to maintenance therapy is associated with a reduced relapse risk

ANCA – antineutrophil cytoplasmic antibody, AAV – antineutrophil cytoplasmic antibody-associated vasculitis, PR3 – proteinase 3, MPO – myeloperoxidase, CYC – cyclophosphamide, AZA – azathioprine, RTX – rituximab, SG – subglottic stenosis