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Abstract
A case of eosinophilic granulomatosis with polyangiitis (EGPA) in which chronic rhinosinusitis (CRS) was improved with a reduction in the myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer after the addition of mepolizumab is reported. A 55-year-old woman with EGPA receiving prednisolone 5 mg/day developed CRS with increases in the eosinophil count and the MPO-ANCA titer. Although it improved with prednisolone 15 mg/day in addition to mizoribine 150 mg/day, because azathioprine could not be taken orally due to side effects, it relapsed after prednisolone was tapered to 5 mg/day. There was no exacerbation of other vasculitis symptoms such as mononeuropathy multiplex. The patient was treated with additional mepolizumab 300 mg every 4 weeks, which resulted in the improvement of CRS and marked reductions of the eosinophil count and MPO-ANCA titer, and the reduction of prednisolone to 2 mg/day. Furthermore, even after tapering mepolizumab to 200 mg every 4 weeks, her condition remained stable without relapse of EGPA and without increases in the eosinophil count and MPO-ANCA titer. The clinical course of mepolizumab treatment in this patient suggests that the IL5-dependent inflammatory cascade is one of the factors contributing to the increase in MPO-ANCA in EGPA.
Highlights
Eosinophilic granulomatosis with polyangiitis (EGPA) has been included in the spectrum of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis [1]
EGPA, which was known as Churg–Strauss syndrome until 2012, is a multisystem disorder that mainly affects the lungs, heart, nasal passages and sinuses, gastrointestinal tract, and skin [2, 12, 15,16,17]. 2 responses and 1/ 17 responses are considered to be involved in the pathogenesis of EGPA [18]. e prominent 2 responses upregulate IL-4, IL-13, and IL-5, and the 1/ 17 responses upregulate interferon (IFN)-c, IL-2, and IL-17, so that eosinophils are activated, they have a long lifespan, and probably cause tissue damage by releasing their granule proteins
Regarding chronic rhinosinusitis (CRS) associated with EGPA, the MIRRA study, a randomized, controlled trial, in 2017 showed mepolizumab reduced the Sino-Nasal Outcome Test-22 (SNOT-22), an indicator of therapeutic effect, and a significant difference between mepolizumab and placebo in the score at weeks 12 and 24 [11, 19, 20]
Summary
Eosinophilic granulomatosis with polyangiitis (EGPA) has been included in the spectrum of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis [1]. EGPA is treated with glucocorticoids and immunosuppressants; most patients remain dependent on glucocorticoid therapy, and relapses are common [6,7,8,9]. Mepolizumab, which is an anti-interleukin (IL)-5 monoclonal antibody, has been approved for the treatment of EGPA. E MIRRA study showed the efficacy and safety of mepolizumab versus placebo as add-on therapy in participants with relapsing or refractory EGPA, resulting in reductions in the glucocorticoid dose [11]. Us, the effect of mepolizumab on MPO-ANCA remains unclear. A case of EGPA in which chronic rhinosinusitis (CRS, considered eosinophilic) was improved with a reduction in the MPO-ANCA titer after the addition of mepolizumab is described
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