Treatment of ANCA-associated vasculitis

@article{Schnermarck2014TreatmentOA,
  title={Treatment of ANCA-associated vasculitis},
  author={Ulf Sch{\"o}nermarck and Wolfgang Ludwig Gross and Kirsten de Groot},
  journal={Nature Reviews Nephrology},
  year={2014},
  volume={10},
  pages={25-36}
}
Antineutrophil cytoplasmic autoantibody (ANCA)-associated diseases are small-vessel vasculitides, encompassing granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Once considered life-threatening diseases, the introduction of stage-adapted immunosuppressive therapy and medications with decreased toxicity has improved patients' survival. Treatment is biphasic, consisting of induction of remission (3–6… 
B cell therapy in ANCA-associated vasculitis: current and emerging treatment options
TLDR
B cell depletion therapy with rituximab is an effective alternative to cyclophosphamide for the induction of remission in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV).
B-cell therapy in antineutrophil cytoplasmic antibody-associated vasculitis.
  • C. Kallenberg, T. Hauser
  • Medicine, Biology
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • 2015
TLDR
To prevent relapses, which occur particularly in patients positive for PR3-ANCA, 500 mg RTX given every 6 months was shown to be superior to azathioprine in a French study, indicating that RTX is a new and promising therapeutic armamentarium for AAV although long-term safety has still to be established.
Full Review B-cell therapy in antineutrophil cytoplasmic antibody-associated vasculitis
TLDR
To prevent relapses, which occur particularly in patients positive for PR3-ANCA, 500 mg RTX given every 6 months was shown to be superior to azathioprine in a French study, indicating that RTX is a new and promising therapeutic armamentarium for AAV although long-term safety has still to be established.
Treatment of renal ANCA-associated vasculitides.
TLDR
This review presents different treatment methods used for remission induction and maintenance in renal AAV, and enumerate the various risk factors associated with relapsing and refractory disease, quality of life impairment and decreased renal and patient survival in AAV.
Strategy of Infection Control in Immunosuppressive Therapy for ANCA-Associated Vasculitis.
TLDR
It will be important for clinicians to observe AAV patients more closely and reduce immunosuppressive drug doses more cautiously, especially to prevent several infections (i.e., deep mycosis, pneumocystis jirovecii pneumonia and cytomegalovirus).
Updates in ANCA-associated vasculitis.
  • C. Pagnoux
  • Biology, Medicine
    European journal of rheumatology
  • 2016
TLDR
The induction treatment for severe granulomatosis with polyangiitis and microscopic polyang iitis is relatively well codified but does not (yet) really differ by precise diagnosis or ANCA type, and the optimal maintenance strategy following rituximab-based induction therapy remains to be determined.
Is B-cell depletion first choice in antineutrophil cytoplasmic antibody-associated vasculitis?
  • C. Kallenberg
  • Medicine, Biology
    Current opinion in rheumatology
  • 2014
TLDR
RTX is an alternative for CYC for induction of remission in generalized AAV and could be first choice for relapsing patients and patients refractory to CYC, but long-term safety should be awaited.
Therapeutic Management of Pediatric Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis
TLDR
Although most of what the authors have learned about pediatric AAV has come from adult data, the quality and breadth of pediatric data is accumulating because of multicenter, international collaborations, and efforts are critical given that optimal treatment strategies likely differ in the context of a developing immune system, and in a physically and emotionally developing child.
Rituximab in relapsing and de novo MPO ANCA-associated vasculitis with severe renal involvement: a case series
TLDR
This study shows a partial efficacy of rituximab in renal function recovery and a low risk of infectious complications in patients with MPO vasculitis with severe renal involvement, in particular in the short term.
Pathogenesis and treatment of ANCA-associated vasculitides.
  • C. Kallenberg
  • Biology, Medicine
    Clinical and experimental rheumatology
  • 2015
TLDR
The B-cell depleting antibody rituximab has been shown non-inferior to cyclophosphamide for induction of remission, and even superior in patients with relapsing disease being positive for PR3-ANCA.
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References

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Complications of long-term therapy for ANCA-associated systemic vasculitis
The antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitides (AASVs) include granulomatosis with polyangiitis and microscopic polyangiitis. These conditions are characterized by
Long-term patient survival in ANCA-associated vasculitis
TLDR
Patients with ANCA-associated vasculitis treated with conventional regimens are at increased risk of death compared with an age- and sex-matched population.
Predictors of Relapse and Treatment Resistance in Antineutrophil Cytoplasmic AntibodyAssociated Small-Vessel Vasculitis
TLDR
A large cohort of patients with ANCA-associated glomerulonephritis and vasculitis was recruited to determine which patients were more likely to relapse; the impact of relapse on long-term outcome; the correlation between length of immunosuppressive therapy and the likelihood of relapse; and the viability of discontinuing immunomodulating therapy in patients who have attained remission.
A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies.
TLDR
In patients with generalized vasculitis, the withdrawal of cyclophosphamide and the substitution of azathioprine after remission did not increase the rate of relapse, suggesting the duration of exposure to cycloph phosphamide may be safely reduced.
Addition of Infliximab to Standard Therapy for ANCA-Associated Vasculitis
TLDR
The addition of infliximab to standard therapy did not confer clinical benefit for patients with active AAV and did not influence remission rates, adverse events, damage index scores, relapse rates or biomarker levels in this cohort study.
A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis.
TLDR
MMF is a reasonable option in the treatment of non-life-threatening recurrent or resistant vasculitis and may obviate the immediate need for recurrent use of cytotoxic agents.
Deoxyspergualin in relapsing and refractory Wegener’s granulomatosis
TLDR
Deoxyspergualin achieved a high rate of disease remission and permitted prednisolone reduction in refractory or relapsing Wegener’s granulomatosis.
Rituximab versus cyclophosphamide for ANCA-associated vasculitis.
TLDR
Rituximab therapy was not inferior to daily cyclophosphamide treatment for induction of remission in severe ANCA-associated vasculitis and may be superior in relapsing disease.
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TLDR
The rates of infection, disease relapse, and death in patients with ANCA small-vessel vasculitis before and after end-stage renal disease (ESRD) in an inception cohort study are described and it is suggested that immunosuppression be geared to patients with ESRD who present with active vasculopathy.
A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody-associated vasculitis.
TLDR
Rituximab was effective remission induction therapy for refractory ANCA-associated vasculitis in this study and there was no difference in efficacy between the 2 main treatment regimens.
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