[Therapy of primary systemic vasculitis].

Area of Science:

• Rheumatology
• Immunology
• Internal Medicine

Context:

• Systemic vasculitides are rare, complex autoimmune diseases.
• Previous treatment relied on broad immunosuppression with cyclophosphamide and prednisolone.
• Multicenter cooperation is crucial for clinical trial assessment due to low disease incidence.

Purpose:

• To outline the evolution of systemic vasculitis treatment over the past decade.
• To describe current stage- and activity-adapted immunosuppressive and immunomodulating therapeutic strategies.
• To differentiate treatment approaches for primary and secondary vasculitides.

Summary:

• Treatment has shifted from a one-size-fits-all immunosuppressive regimen to a more personalized approach.
• Induction therapy for small/medium vessel vasculitis involves methotrexate or cyclophosphamide, with plasma exchange for severe cases, alongside prednisolone.
• Maintenance therapy utilizes less potent agents like azathioprine for long-term tolerability.
• Large vessel vasculitis primarily uses glucocorticoids, with azathioprine or methotrexate as adjuncts.
• Secondary vasculitides require treating the underlying cause, with immunosuppression reserved for severe manifestations.

Impact:

• The shift towards differentiated therapy aims to optimize treatment efficacy while minimizing long-term side effects.
• Personalized treatment strategies enhance patient management for various systemic vasculitides.
• Improved understanding and application of immunomodulating agents contribute to better disease control and quality of life.