Therapeutic target
- Risk reduction or prevention of complications.
Therapy recommendations
Below are treatment recommendations for the most common vasculitides.
- Anti-GBM (glomerular basement membrane) disease, formerly Goodpasture’s syndrome:
- High-dose glucocorticoids (steroids) and cyclophosphamide (alkylants).
- Plasmapheresis (plasma exchange) – to remove the antibodies.
- Duration of therapy: 8-12 months
- Eosinophilic granulomatosis with polyangiitis, formerly Churg-Strauss syndrome:
- Therapy is based on the number of eosinophils (should be less than 700/ml).
- Cortisone therapy alone is indicated in the absence of cardiac involvement or severe inflammation of the peripheral nervous system.
- In case of manifestation in the heart, kidneys, CNS use of immunosuppressants.
- Granulomatosis with polyangiitis, formerly Wegener’s granulomatosis:
- Therapy is stage- and activity-based.
- Induction therapy: treatment with glucocorticoids (steroids), methotrexate (MTX) (folic acid antagonist/immunosuppressants), cyclophosphamide (alkylants).
- Maintenance therapy
- Once remission occurs, therapy is switched to azathioprine (24 months) or leflunomide (immunosuppressant) or methotrexate. In addition, prednisolone.
- Rituximab at reduced dose (500 mg every 6 months) is superior to azathioprine in maintenance therapy after cyclophosphamide induction.
- Second-line alternatives: leflunomide and mycophenolate mofetil.
- Reserve drugs: rituximab (monoclonal antibody; recommended for induction therapy alongside cyclophosphamide in patients with critical organ involvement), infliximab (TNF-alpha blocker).
- Therapy is stage- and activity-based.
- Isolated leukocytoclastic skin vasculitis: treatment with prednisolone equivalent (glucocorticoids).
- Kawasaki syndrome (MCLS): treatment with immunoglobulins, acetylsalicylic acid (platelet aggregation inhibitor), and infliximab (TNF-alpha blocker) if resistant to immunoglobulins.
- Microscopic polyangiitis:
- Therapy is stage- and activity-based.
- Induction therapy: treatment with glucocorticoids (steroids), methotrexate (MTX) (folic acid antagonist/immunosuppressants), cyclophosphamide (alkylants).
- Maintenance therapy
- Once remission occurs, therapy is switched to azathioprine (24 months) or leflunomide (immunosuppressant) or methotrexate. In addition, prednisolone.
- Rituximab at reduced dose (500 mg every 6 months) is superior to azathioprine in maintenance therapy after cyclophosphamide induction.
- Second-line alternatives: leflunomide and mycophenolate mofetil.
- Reserve drugs: rituximab (monoclonal antibody; recommended for induction therapy alongside cyclophosphamide in patients with critical organ involvement), infliximab (TNF-alpha blocker).
- Therapy is stage- and activity-based.
- Polyarteritis nodosa (PAN; panarteritis nodosa): treatment with prednisolone equivalent (glucocorticoids), in mild course as monotherapy; cyclophosphamide (alkylants).
- Purpura Schoenlein-Henoch: treatment with prednisolone equivalent (glucocorticoids).
Note: The German Society of Rheumatology (DGRh) has advocated the use of the monoclonal antibody rituximab (RTX) in ANCA-associated vasculitides (AAV). Accordingly, rituximab would be an alternative to cyclophosphamide (see below) the current standard of care for AAV.
For more detailed disease-related therapy recommendations, see the corresponding disease.