Data synthesis: We included 2 meta-analyses, 20 randomized controlled prospective trials, and 62 uncontrolled trials with more than 10 patients or observational studies.
Outcome measures and treatment protocols were heterogeneous across trials. Cotrimoxazole can be used alone or in combination with corticosteroids to induce and maintain remission in cases of isolated upper respiratory tract involvement. To induce remission, methotrexate plus corticosteroids can be used instead of cyclophosphamide for patients with generalized, non-organ-threatening disease.
When methotrexate is used as maintenance therapy, the likelihood of relapse is high and rigorous monitoring is mandatory. Rituximab should be considered as the first-choice agent for maintenance of remission; azathioprine and methotrexate are appropriate alternatives if rituximab is unavailable or contraindicated. Longer treatment duration with rituximab for maintenance of remission reduces relapses in severe GPA and MPA, but it is unclear to date whether all patients, or only some subsets, would benefit of 4-year duration of repeated, systematic RTX infusions.
The role of low-dose prednisone in maintenance of remission is not definitely demonstrated or established ongoing studies. Makhzoum reports personal fees from Hoffmann-La Roche, outside the submitted work. The other authors have no conflicts of interest.
National Center for Biotechnology Information , U. Journal List Int J Rheumatol v. Int J Rheumatol. Published online Apr 8. Karla N. Author information Article notes Copyright and License information Disclaimer.
Corresponding author. Jean-Paul Makhzoum: ac. Samman et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Associated Data Data Availability Statement All data presented in this review article has been published as original articles or abstracts and available online. Abstract Significant progress has been made in the treatment of ANCA-associated vasculitides AAV , notably in granulomatosis with polyangiitis and microscopic polyangiitis. Introduction Vasculitides are classified according to the size of vessels predominantly involved in the inflammatory process [ 1 ].
Classification of Disease Severity In practice, AAV are clinically classified according to the severity nonsevere or severe and the extent limited or systemic of the disease [ 9 ]. Open in a separate window. Figure 1. Figure 2. Avacopan The central role of neutrophil activation in the pathogenesis of AAV is well described [ 4 ]. Mycophenolate Mofetil MMF, an immunosuppressant that acts selectively on lymphocytes, is used for various autoimmune diseases and for organ transplantation [ 41 ].
Maintenance of Remission Therapy Once remission is achieved, maintenance of remission Figure 3 is important to prevent disease relapses.
Figure 3. Take-Home Messages 7. Induction of Remission First-line agents for induction of remission in severe GPA and MPA include cyclophosphamide or rituximab, along with glucocorticoids Rapid reduction of glucocorticoids is safe and should be considered for induction of remission in severe GPA and MPA Avacopan, if confirmed safe and cost-effective, may become an alternative to GC and lead to a GC-free regimen of AAV Plasma exchange is no longer routinely recommended in most patients with severe GPA and MPA; it has not been proven effective in terms of survival and reduction of ESRD risk in patients with severe disease.
Maintenance of Remission Rituximab should be considered as the first-choice agent for maintenance of remission; azathioprine and methotrexate are appropriate alternatives if rituximab is unavailable or contraindicated Longer treatment duration with rituximab for maintenance of remission reduces relapses in severe GPA and MPA, but it is unclear to date whether all patients, or only some subsets, would benefit of 4-year duration of repeated, systematic RTX infusions Leflunomide and mycophenolate mofetil can be used when RTX, MTX, and AZA are contraindicated or not tolerated The role of low-dose prednisone in maintenance of remission is not definitely demonstrated or established ongoing studies.
Data Availability All data presented in this review article has been published as original articles or abstracts and available online. Conflicts of Interest Dr. References 1. Jennette J. Arthritis and Rheumatism. Zarka F. A review of primary vasculitis mimickers based on the Chapel Hill Consensus classification. International Journal of Rheumatology.
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Milman N. Increasing the efficacy of CD20 antibody therapy through the engineering of a new type II anti-CD20 antibody with enhanced direct and immune effector cell—mediated B-cell cytotoxicity.
Furie R. McClure M. Atisha-Fregoso Y. Popa E. Allergy Clin. Gatto D. The germinal center reaction. Langford C. Karnell J. Drug Deliv. Corneth O. Von Borstel A. The SYK tyrosine kinase: A crucial player in diverse biological functions. Spleen tyrosine kinase inhibition is an effective treatment for established vasculitis in a pre-clinical model. Kusunoki Y. Support Center Support Center. External link. Please review our privacy policy. Nose and sinuses, lungs, kidneys, joints, eyes.
Lungs, upper airways, peripheral nerves, heart, skin. Not fulfilling any of the above criteria. No difference in time to remission.
Higher risk of relapse in the pulse CYC group on long-term follow up [ ]. MEPEX [ ]. Renal recovery at 3 months. PLEX superior. No difference in long-term outcomes [ ]. No difference 2 No difference in efficacy. No difference in subgroup analysis for ESRD, death, alveolar hemorrhage. RAVE [ ]. RTX better for relapsing disease. DO CYC 1. Relapse at 18 months.
Pulse CYC. RTX mg days 0, 14 then every 6 months. Major organ relapse at 28 months. Most relapses occurred after B-cell return. Scheduled RTX mg every 6 months. Relapse at 28 months. No difference in adverse events. Vasculitis Foundation P. Do you know of an organization? Living With Living With. Financial Resources The HealthWell Foundation provides financial assistance for underinsured patients living with chronic and life-altering conditions.
They offer help with drug copayments, deductibles, and health insurance premiums for patients with specific diseases. The disease fund status can change over time, so you may need to check back if funds are not currently available.
Learn More Learn More. In-Depth Information Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. Have a question? References References. Recent advances in anti-neutrophil cytoplasmic antibody-associated vasculitis. Indian Journal of Nephrology.
Antineutrophil Cytoplasmic Antibody-Associated Vasculitides. Is It Time to Split up the Group?. Ann Rheum Dis.
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