Related Experiment Video
Updated: Dec 13, 2025

Catheter-based Endovascular Angioplasty for Fibrosing Mediastinitis-associated Pulmonary Vein Stenosis
Published on: August 26, 2025
New therapeutic strategies in lung vasculitis
Mouhamad Nasser1, Vincent Cottin1,2
1Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon.
Purpose Of Review:
To summarize the latest publications and provide a practical overview of treatment strategies for lung vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs).
Recent Findings:
In patients with severe ANCA-associated vasculitis, plasma exchange, as adjunctive therapy to standard treatment, is not associated with improved survival or reduced risk of end-stage kidney disease. A regimen with reduced dose of glucocorticoids is equally effective to induce remission as a standard regimen. In patients without organ or life-threatening disease, mycophenolate mofetil can be used in combination with oral glucocorticoid therapy to induce remission, however, with a higher risk of relapse than when using rituximab or cyclophosphamide. For maintenance of remission, a tailored regimen of rituximab infusion was equivalent to a fixed regimen, with fewer perfusions. Belimumab, a human IgG1(Equation is included in full-text article.)monoclonal antibody against B-lymphocyte stimulator, did not decrease the relapse rate when added to azathioprine and glucocorticoids. Avacopan, a complement C5a receptor inhibitor, was effective in replacing high-dose glucocorticoids in achieving complete remission of vasculitis.
Summary:
Significant advances have been made in the treatment strategy to both induce remission and maintain remission in patients with ANCA-associated vasculitis. The choice should take into consideration efficacy, cost-effectiveness, safety profile, ease of use, and possibility of individual tailoring of treatment.
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