Induction and Maintenance Therapy in Vasculitis

Management of systemic vasculitis is conventionally framed in two phases: remission induction, which uses more intensive immunosuppression to bring active disease under control, and remission maintenance, which uses less intensive, longer-term therapy to prevent relapse. This two-phase structure is most fully developed for ANCA-associated vasculitis, where randomised trials have defined the principal therapeutic options.

Definition

Induction and maintenance therapy is the two-phase treatment strategy for systemic vasculitis in which intensive immunosuppression is first used to induce remission of active disease, followed by less intensive therapy to maintain remission and prevent relapse.

Scope

The entry explains the conceptual division between induction and maintenance, the rationale for sequencing therapy this way, and the categories of evidence that inform it — including landmark randomised trials in ANCA-associated vasculitis and EULAR recommendations. It describes therapeutic strategy at the level of principles and study evidence; it deliberately avoids drug dosing and individualised treatment advice, and is not a clinical entity.

Core questions

• Why is vasculitis treatment divided into induction and maintenance phases?
• What categories of agent have been studied for remission induction in ANCA-associated vasculitis?
• What is the role of plasma exchange and adjunctive measures in severe disease, according to trials?
• How do guidelines translate trial evidence into a phased strategy?

Key concepts

• Remission induction phase
• Remission maintenance phase
• Glucocorticoids as backbone therapy
• Cyclophosphamide and rituximab for induction
• Relapse prevention
• Plasma exchange in severe disease
• Disease severity stratification
• Guideline-based treat-to-target framing

Mechanisms

The phased approach reflects the biology of relapsing autoimmune disease: active vasculitis requires rapid, intensive immunosuppression to halt vessel injury (induction), after which the immunosuppressive burden is reduced to a level that suppresses relapse with fewer cumulative toxicities (maintenance). In ANCA-associated vasculitis, randomised trials compared rituximab with cyclophosphamide for induction (the RAVE and RITUXVAS trials) and evaluated the role of adjunctive plasma exchange and glucocorticoid regimens in severe disease (the PEXIVAS trial). Maintenance then relies on lower-intensity regimens aimed at preventing the relapses that characterise these diseases.

Clinical relevance

The induction-maintenance framework organises how the vasculitis treatment literature and guidelines are structured, and understanding it helps in appraising trial evidence and recommendations. This entry presents that framework and its evidence base for reference and education; it is non-prescriptive, omits dosing, and is not a basis for individual treatment decisions, which require specialist clinical judgement.

Evidence & guidelines

The strategy rests on randomised trials in ANCA-associated vasculitis — including comparisons of rituximab and cyclophosphamide for induction and evaluation of plasma exchange and glucocorticoid regimens in severe disease — and is codified in the EULAR recommendations for the management of ANCA-associated vasculitis. For large vessel vasculitis, the EULAR large vessel vasculitis recommendations provide an analogous evidence-based framework. These sources frame the phased approach described here.

History

Cyclophosphamide plus glucocorticoids transformed previously fatal systemic vasculitis into a treatable, relapsing condition from the 1970s onward, establishing the induction-maintenance paradigm. Randomised trials in the 2000s and 2010s — notably RAVE and RITUXVAS for rituximab-based induction and PEXIVAS for plasma exchange and glucocorticoid dosing — refined the strategy, which successive EULAR recommendations have consolidated.

Debates

What is the role of plasma exchange in severe ANCA-associated vasculitis?

The PEXIVAS trial did not show that adjunctive plasma exchange reduced the composite of death or end-stage kidney disease overall, prompting a more selective view of its use and influencing how guidelines position it within induction strategy.

Key figures

• John H. Stone
• David R. W. Jayne
• Peter A. Merkel
• Bernhard Hellmich
• Ulrich Specks

Seminal works

• stone-2010
• jones-2010
• walsh-2020
• hellmich-2024

Frequently asked questions

Why is vasculitis treatment split into induction and maintenance?

Because active disease needs intensive immunosuppression to be brought under control (induction), after which lower-intensity therapy is used to keep the disease in remission and prevent relapse while limiting cumulative toxicity (maintenance).

Which trials shaped induction therapy in ANCA-associated vasculitis?

Randomised trials including RAVE and RITUXVAS compared rituximab with cyclophosphamide for remission induction, and PEXIVAS evaluated plasma exchange and glucocorticoid regimens in severe disease; their results inform current EULAR recommendations.