The Recommendations for the Management of ANCA-Associated Vasculitis(Part 4)
We continue this week, our discussion of the recommendations on the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Last week, we ended our discussion on the classic treatment to contain the disease’s progression.Experts encourage prophylaxis against infection with Pneumocystis jirovecii with trimethoprim/ sulfamethoxazole (800/160 mg on alternate days or 400/80 mg daily) in all patients being treated with cyclophosphamide, if not contraindicated.
Using inhaled monthly pentamidine, if there is an adverse reaction or contraindication to trimethoprim/sulfamethoxazole, may be useful but is not known to be cost-effective and not usuallyused.
Alternative treatments may include dapsone and atovaquone.
Rituximab in AAV has been tested in two (2) randomized controlled trials (RCTs) for the treatment of Wegener’s Granulomatosis and Microscopic Polyangiitis) the randomized open label trial comparing a rituximab based treatment with a standard cyclophosphamide/azathioprine therapy in the treatment of active, ‘generalized’ AAV.
In the 2 RCTs, patients first took high-dose glucocorticoids with the subsequent dose tapered.
The rituximab dose in both studies was given once for four infusions.
In both RCTs, rituximab was equal in effect to cyclophosphamide and shown to be more effective to prevent relapse.
The grade of evidence for the use of rituximab in patients with eosinophilic granulomatosis with polyangiitis (EGPA) is lower than for polyangiitis (GPA)/ microscopic polyangitis (MPA) patienfs.
An analysis of 41 patients with EGPA who took different treatment of rituximab showed that 34% reached complete remission at 6 months and complete remission for 49% of patients at 12 months.
As this is quite expensive, rituximab is restricted in some countries as such expert centers are considered.
In some instances, rituximab is chosen over cyclophosphamide, especially in patients who want to preserve their ability to reproduce.
Cyclophosphamide is linked with lowered ovarian reserve, ovarian failure and possibly male infertility.
However, the long-term impact of rituximab on the reproduction ability has not been studied.
Treatment in patients with severe AAV, should not be delayed but there is a need to discuss the issues.
The AAVs have manifestations and the spectrum of disease range from the indolent to the life-threatening.