Treatment Algorithm for the Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitides (AAV) - Flowchart
Treatment Algorithm for the Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitides (AAV) - Flowchart ANCA-Associated Vasculitis ANCA-Associated Vasculitis
«Flowchart»

Active AAV

Active AAV

Active AAV

EGPA without poor prognostic factors

EGPA without poor prognostic factors

EGPA without poor prognostic factors

EGPA with poor prognostic factors

EGPA with poor prognostic factors

EGPA with poor prognostic factors

Nonorgan-threatening GPA or MPA

Nonorgan-threatening GPA or MPA

Nonorgan-threatening GPA or MPA

Systemic or organ-threatening disease (GPA, MPA)

Systemic or organ-threatening disease (GPA, MPA)

Systemic or organ-threatening disease (GPA, MPA)

Mepolizumab or unproven therapies2 with or without steroids

Mepolizumab or unproven therapies2 with or without steroids

Mepolizumab or unproven therapies2 with or without steroids

2 2

Low-dose steroids with or without steroid-sparing agent if needed

Low-dose steroids with or without steroid-sparing agent if needed

Low-dose steroids with or without steroid-sparing agent if needed

Steroids

Steroids

Steroids

Remission

Remission

Remission

Refractory disease

Refractory disease

Refractory disease

End

End

End

Mepolizumab, or consider unproven therapies2

Mepolizumab, or consider unproven therapies2

Mepolizumab, or consider unproven therapies2

2 2

MTX1 or AZA1 with or without low-dose steroids

MTX1 or AZA1 with or without low-dose steroids

MTX1 or AZA1 with or without low-dose steroids

1 1 1 1

CYC with steroids

CYC with steroids

CYC with steroids

Remission

Remission

Remission

Refractory disease

Refractory disease

Refractory disease

Consider RTX, AZA, or CYC with steroids

Consider RTX, AZA, or CYC with steroids

Consider RTX, AZA, or CYC with steroids

MTX with or without low-dose steroids

MTX with or without low-dose steroids

MTX with or without low-dose steroids

MTX with steroids

MTX with steroids

MTX with steroids

Remission

Remission

Remission

Refractory disease

Refractory disease

Refractory disease

CYC or RTX depending on initial agent chosen, otherwise consider unproven therapies3

CYC or RTX depending on initial agent chosen, otherwise consider unproven therapies3

CYC or RTX depending on initial agent chosen, otherwise consider unproven therapies3

3 3

RTX, MTX, or AZA with or without low-dose steroids

RTX, MTX, or AZA with or without low-dose steroids

RTX, MTX, or AZA with or without low-dose steroids

CYC or RTX with steroids

CYC or RTX with steroids

CYC or RTX with steroids

Remission

Remission

Remission

Refractory disease

Refractory disease

Refractory disease

1Widely used but without strong evidence of efficacy

1Widely used but without strong evidence of efficacy

1Widely used but without strong evidence of efficacy

1

2Includes RTX, hydroxurea, MTX, AZA, and MMF

2Includes RTX, hydroxurea, MTX, AZA, and MMF

2Includes RTX, hydroxurea, MTX, AZA, and MMF

2

3Includes infliximab, MMF, intravenous immunoglobulin, and 15-deoxyspergualin

3Includes infliximab, MMF, intravenous immunoglobulin, and 15-deoxyspergualin

3Includes infliximab, MMF, intravenous immunoglobulin, and 15-deoxyspergualin

3