In 2020 a number of clinical trials have provided insights into therapeutic approaches for the treatment of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis and lupus nephritis. Moreover, mechanistic insights have potential to open new therapeutic strategies in the future.
Key advances
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The addition of plasma exchange to standard of care induction therapy is not associated with improved outcomes in most patients with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV); the use of a reduced-dose glucocorticoid regimen is non-inferior to standard dose for remission induction, but is associated with a significantly reduced risk of serious infections1.
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Therapy with rituximab is effective in inducing remission in patients with relapsing AAV2.
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Direct inhibition of myeloperoxidase is associated with attenuated inflammatory pathways in vitro and reduced kidney injury in a mouse model of nephrotoxin-induced nephropathy4.
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The addition of intravenous belimumab to mycophenolate mofetil induction therapy modestly improved outcomes in a selected population with lupus nephritis5.
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Sequential combination of rituximab followed by belimumab in lupus nephritis reduced the return of autoreactive B cells7.
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B cell specific Toll-like receptor 9 regulates kidney disease in a mouse model of systemic lupus erythematosus and could be a therapeutic target9.
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References
Walsh, M., Merkel, P. A. & Peh, C. A. et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N. Engl. J. Med. 382, 622–631 (2020).
Smith, R. M., Jones, R. B. & Specks, U. et al. Rituximab as therapy to induce remission after relapse in ANCA-associated vasculitis. Ann. Rheum. Dis. 79, 1243–1249 (2020).
Stone, J. H., Merkel, P. A. & Spiera, R. et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N. Engl. J. Med. 363, 221–232 (2010).
Antonelou, M., Michaelsson, E. & Evans, R. D. R. et al. Therapeutic myeloperoxidase inhibition attenuates neutrophil activation, ANCA-mediated endothelial damage, and crescentic GN. J. Am. Soc. Nephrol. 31, 350–364 (2020).
Furie, R., Rovin, B. H. & Houssiau, F. et al. Two-Year, randomized, controlled trial of belimumab in lupus nephritis. N. Engl. J. Med. 383, 1117–1128 (2020).
Jackson, S. W. & Davidson, A. BAFF inhibition in SLE-Is tolerance restored? Immunol. Rev. 292, 102–119 (2019).
Atisha-Fregoso, Y., Malkiel, S. & Harris, K. M. et al. CALIBRATE: a phase 2 randomized trial of rituximab plus cyclophosphamide followed by belimumab for the treatment of lupus nephritis. Arthritis & Rheumatology https://doi.org/10.1002/art.41466 (2020).
Nickerson, K. M., Wang, Y., Bastacky, S. & Shlomchik, M. J. Toll-like receptor 9 suppresses lupus disease in Fas-sufficient MRL Mice. PLoS One. 12, e0173471 (2017).
Tilstra, J. S., John, S. & Gordon, R. A. et al. B cell-intrinsic TLR9 expression is protective in murine lupus. J. Clin. Invest. 130, 3172–3187 (2020).
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Competing interests
P.H.N. was an investigator in the PEXIVAS and RITAZAREM trials and is a lead investigator in the Belimumab with Rituximab for Primary Membranous Nephropathy (REBOOT) trial (NCT03949855), which is sponsored by the Immune Tolerance Network. He was an investigator in trials of AAV sponsored by Chemocentryx and InflaRx, and has served as an ad hoc advisor for Chemocentryx. I.A. declares no competing interests.
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Ayoub, I., Nachman, P.H. Advances in ANCA-associated vasculitis and lupus nephritis. Nat Rev Nephrol 17, 89–90 (2021). https://doi.org/10.1038/s41581-020-00388-x
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DOI: https://doi.org/10.1038/s41581-020-00388-x