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Published on: February 8, 2019
[Wegener's granulomatosis and microscopic polyangiitis]
K de Groot1, E Reinhold-Keller
1Medizinische Klinik III (Innere Medizin, Nephrologie, Rheumatologie), Klinikum Offenbach GmbH, Starkenburgring 66, 63069, Offenbach. kirsten@de-groot.de
Abstract:
Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are primary systemic small vessel vasculitides, associated with a positive C/PR3-ANCA in WG and P/MPO-ANCA in MPA. The most prominently involved organs are the upper (only in WG) and lower respiratory tract and the kidneys. The diagnostic work-up is an interdisciplinary approach assessing disease stage and extent. Treatment is adapted to disease stage and extent and relies on a combination of a cytotoxic plus a tapering regimen of corticosteroids. Induction of remission in "early systemic" disease can be achieved with low-dose methotrexate. In severe generalized vasculitis cyclophosphamide (CYC) is the mainstay of therapy, in rapidly progressive glomerulonephritis in combination with plasmapheresis. After 3-6 months of induction CYC is switched to a maintenance treatment with azathioprine. Alternatives are leflunomide, mycophenolate or methotrexate (creatinine < 150 micromol/l). Age > or = 50 at diagnosis, renal dysfunction and pulmonary involvement are associated with higher mortality rates. The relapse rate is approximately 50% within 5 years, being higher in WG than MPA.
Insights
Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) are small vessel vasculitides diagnosed via ANCA testing. Treatment involves immunosuppressants and corticosteroids, with relapse rates around 50% within five years.
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.
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