From the FDA Drug Label
The main outcome measure for both GPA and MPA patients was achievement of complete remission at 6 months defined as a BVAS/GPA of 0, and off glucocorticoid therapy A majority of the patients were ANCA-positive. The answer to the question of Scleritis with c-ANCA positive is not directly addressed in the provided drug labels. Key points:
- The labels discuss the treatment of Granulomatosis with Polyangiitis (GPA) and Microscopic Polyangiitis (MPA), which are associated with ANCA positivity.
- However, they do not specifically mention Scleritis.
- Therefore, no conclusion can be drawn about the treatment of Scleritis with c-ANCA positive based on the provided information. The FDA drug label does not answer the question.
From the Research
Scleritis with positive c-ANCA should be treated with rituximab and high-dose corticosteroids, as this combination has shown to be effective in achieving remission and maintaining it, as seen in a recent case report 1. The treatment of scleritis with positive c-ANCA requires prompt systemic immunosuppression to prevent permanent vision loss and potentially life-threatening systemic vasculitis.
- High-dose corticosteroids, such as prednisone 1 mg/kg/day, with a maximum of 60-80 mg daily, should be initiated immediately.
- Rituximab, 375 mg/m² weekly for 4 weeks, can be used as a first-line treatment, especially in patients with severe disease threatening vision, as it has been shown to be effective in achieving remission and maintaining it 1.
- For patients who do not respond to rituximab, cyclophosphamide (2 mg/kg/day orally) can be considered as an alternative.
- Once remission is achieved, typically within 3-6 months, maintenance therapy with less toxic agents like azathioprine (2 mg/kg/day), methotrexate (15-25 mg weekly), or mycophenolate mofetil (1-2 g/day) should be continued for at least 18-24 months.
- Regular monitoring of inflammatory markers, kidney function, and complete blood counts is essential to prevent complications and adjust treatment as needed.
- Pneumocystis pneumonia prophylaxis with trimethoprim-sulfamethoxazole is recommended during immunosuppression to prevent opportunistic infections. The positive c-ANCA indicates autoantibodies (typically against proteinase-3) that activate neutrophils, causing blood vessel inflammation and tissue damage, and treatment should be tailored to address this underlying mechanism, as supported by recent studies 2, 3, 4.