Update on maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis

  title={Update on maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis},
  author={Ora Singer and William J. Mccune},
  journal={Current Opinion in Rheumatology},
Purpose of review The antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitides are a group of rare systemic diseases. The past several years have seen major therapeutic advances in the treatment of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The success rate in induction of remission is high, but reducing the high incidence of relapses remains a therapeutic challenge. Recent findings Studies have shown no improvement in relapse rates in GPA and MPA… 
Granulomatosis with polyangiitis
In ANCA-associated vasculitis in remission, tailored vs fixed-schedule rituximab did not differ for relapse at 28 months
The MAINRITSAN2 trial found that rituximab, 500 mg on days 0 and 14 and at months 6, 12, and 18, was more effective than daily oral azathioprine and groups did not differ for relapse at 28 months.
Granulomatosis with Polyangiitis (Wegener's Granulomatosis): Evolving Concepts in Treatment.
Rituximab (RTX), a monoclonal antibody directed against B cells, has been shown to be at least as effective (and possibly more effective) as CYC, and the use of RTX may reduce the need for maintenance immunosuppression.
Anti-cytokine targeted therapies for ANCA-associated vasculitis.
Assessment of the benefits and harms of anti-cytokine targeted therapy for adults with AAV found low-certainty evidence suggests that more participants in the mepolizumab group had ≥ 24 weeks of accrued remission over 52 weeks compared to placebo, while three studies were at high risk and one study was at unclear risk of bias for selective outcome reporting.
Chronic Pulmonary Aspergillosis and Type 2 Diabetes Mellitus Complicating Granulomatosis with Polyangiitis in an Adult Saudi Male: A Case Report
The clinical and radiological features of GPA are similar to those of pulmonary tuberculosis, chronic pulmonary aspergillosis, and lung cancer, so a high degree of suspicion is required to make the correct diagnosis.
Granulomatosis with Polyangitis (GPA)
A 43-year-old lady presenting with longstanding bilateral nasal obstruction, anosmia, bloody crusts and blood-stained nasal discharge. She also had bilateral hearing loss. She tried some topical
Granulomatose com poliangiite
A granulomatose com poliangiite e uma entidade de elevada morbimortalidade, e teve seu algoritmo de tratamento recentemente atualizado, conforme abordado nesta revisao.


Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis.
More patients with ANCA-associated vasculitides had sustained remission at month 28 with rituximab than with azathioprine, and the frequencies of severe adverse events were similar in the two groups.
Efficacy and safety of rituximab as maintenance therapy for relapsing granulomatosis with polyangiitis—a case series
Study of pre-emptive rituximab (RTX) in a series of patients with severe relapsing granulomatosis with polyangiitis found it to be an effective maintenance treatment in a population with severe, relapsesing long-standing GPA.
A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies.
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.
Azathioprine or methotrexate maintenance for ANCA-associated vasculitis.
The two agents appear to be similar alternatives for maintenance therapy in patients with Wegener's granulomatosis and microscopic polyangiitis after initial remission, and the primary hypothesis that methotrexate is safer than azathioprine is not supported.
Rituximab versus cyclophosphamide for ANCA-associated vasculitis.
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.
Rituximab for remission induction and maintenance in refractory granulomatosis with polyangiitis (Wegener's): ten-year experience at a single center.
RTX appeared to be effective and safe for the induction and maintenance of remission in patients with chronic relapsing GPA, and Repeated depletion of B lymphocytes seems to be associated with a low risk of infections.
Ofatumumab for B cell depletion therapy in ANCA-associated vasculitis: a single-centre case series
Treatment with ofatumumab resulted in similar serological and clinical responses to those seen in previous cohorts treated at this centre with a comparable CS, CYC and rituximab-based regimen, and should be considered an alternative B cell depleting agent in patients who are intolerant of, or unresponsive to, ritUXimab.
Rituximab for refractory granulomatosis with polyangiitis (Wegener's granulomatosis): comparison of efficacy in granulomatous versus vasculitic manifestations
The overall response rate of refractory GPA to RTX was high, and response rates of vasculitic manifestations were excellent; failure of response/progress was mostly due to granulomatous manifestations, especially orbital masses.
Rituximab for remission maintenance in relapsing antineutrophil cytoplasmic antibody-associated vasculitis.
In the absence of biomarkers that accurately predict relapse, routine rituximab re-treatment may be an effective strategy for remission maintenance in patients with refractory and relapsing AAV.