[Wegener's granulomatosis and microscopic polyangiitis]

Area of Science:

• Rheumatology
• Immunology
• Nephrology

Context:

• Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are primary systemic small vessel vasculitides.
• These conditions are associated with specific autoantibodies: C/PR3-ANCA in WG and P/MPO-ANCA in MPA.
• Key organs affected include the respiratory tract and kidneys.

Purpose:

• To outline the diagnostic and therapeutic strategies for WG and MPA.
• To highlight factors influencing prognosis and relapse rates.

Summary:

• Diagnosis requires an interdisciplinary approach to assess disease stage and extent.
• Treatment involves cytotoxic agents and corticosteroids, tailored to disease severity.
• Remission induction for early disease may use methotrexate; severe cases often require cyclophosphamide, potentially with plasmapheresis.
• Maintenance therapy typically involves azathioprine, with alternatives like leflunomide, mycophenolate, or methotrexate.
• Age over 50, renal dysfunction, and pulmonary involvement are linked to increased mortality.

Impact:

• Understanding these vasculitides is crucial for timely diagnosis and effective management.
• Identifying prognostic factors aids in patient stratification and treatment intensity.
• High relapse rates necessitate long-term monitoring and management strategies.