Familial Eosinophilic Granulomatosis with Polyangiitis in a Sister and Brother

Ueki, Yuriko; Oshikata, Chiyako; Asai, Yoshihito; Kaneko, Takeshi; Tsurikisawa, Naomi

doi:10.2169/internalmedicine.3744-19

Cited by 7 publications

(5 citation statements)

References 18 publications

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“…Although IVIG has been used effectively to treat peripheral neuropathy and cardiac dysfunction in patients with EGPA [ 8 ], our review of the literature did not yield any reports regarding its effectiveness in patients with optic neuritis. However, IVIG has been shown to be effective in neuromyelitis optica [ 9 ], facial paralysis [ 10 ], and vasculitis-associated cerebral infarction [ 11 ]. In patients with facial paralysis and cerebral infarction, their symptoms resolved only after IVIG was added to steroids and cyclophosphamide.…”

Section: Discussionmentioning

confidence: 99%

Intravenous cyclophosphamide and immunoglobulin ameliorated visual field defects in a patient with eosinophilic granulomatosis with polyangiitis

Motobayashi,

Oshikata,

Kodama

et al. 2024

Respiratory Medicine Case Reports

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Section: Discussionmentioning

confidence: 99%

Intravenous cyclophosphamide and immunoglobulin ameliorated visual field defects in a patient with eosinophilic granulomatosis with polyangiitis

Motobayashi,

Oshikata,

Kodama

et al. 2024

Respiratory Medicine Case Reports

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“…MIRRA was the rst randomized controlled trial of mepolizumab for treating refractory or relapsing EGPA [25]. Other published studies support the use of mepolizumab for the induction and maintenance of remission in refractory, relapsing, or glucocorticoid-dependent EGPA [25,[50][51][52][53]. However, it has not been conclusively determined whether mepolizumab treatment is effective against various vasculitis signs in EGPA or ANCA-positive cases [54].…”

Section: Discussionmentioning

confidence: 99%

“…There are reports that mepolizumab is effective for vascular symptoms in EGPA patients [21,25,[50][51][52][53]57], but there are no reports regarding its effect on long-term prognosis. In the super-responder group, the relapse rate was signi cantly lower in the 3rd year and at the last visit (mean and standard deviation of 4.3 ± 1.0 years after the start of mepolizumab) compared with the 1st year after the start of mepolizumab administration (Table 2).…”

Section: Discussionmentioning

confidence: 99%

Long-term treatment by mepolizumab reduces the relapse rate in patients with eosinophilic granulomatosis with polyangiitis

Masumoto

Oshikata²,

Nakadegawa

et al. 2023

Preprint

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Background: The mainstay of treatment for eosinophilic granulomatosis with polyangiitis (EGPA) is systemic corticosteroid therapy; some patients also receive intravenous immunoglobulins, other immunosuppressive agents, and biologics. Mepolizumab, an anti-interleukin-5 monoclonal antibody, in EGPA patients induces remission and decreases the daily dose of corticosteroids; however, the prognosis of long-term mepolizumab treatment for EGPA and its clinical efficacy are unknown. Methods: Seventy-one EGPA patients were treated at Hiratsuka City Hospital, Japan, between April 2018 and March 2022. We administered mepolizumab for mean 2.8±1.7 years to 43 patients in whom remission could not be induced by conventional treatment. After excluding 18 patients who received mepolizumab for less than 3 years, we classified 15 patients in the “super-responder group” (the daily dose of corticosteroids or another immunosuppressant could be decreased, or the interval between IVIG treatments could be prolonged) and 10 patients in the “responder group” (neither of these changes could be achieved). Eosinophil numbers, serum IgG levels, daily doses of corticosteroids and other immunosuppressants, the Birmingham Vasculitis Activity Score (BVAS), and relapse frequency before and after mepolizumab initiation were determined. Results: Eosinophil numbers at diagnosis or the lowest serum IgG level before mepolizumab treatment were higher in the super-responder group than in the responder group (p < 0.05). In the super-responder group, the prednisolone dose at last visit after mepolizumab initiation was lower than before treatment (p < 0.01) and in the responder group (p < 0.01). In both groups, the number of peripheral blood eosinophils and BVAS decreased after starting mepolizumab compared to before treatment (P < 0.01). BVAS before mepolizumab (p < 0.05) and at the last visit (p < 0.01) in the super-responder group were lower than in the responder group. Relapse rates every year after the start of mepolizumab were lower in the super-responder group than in the responder group (p <0.01). Relapse rates decreased during the 3 years following initiation of mepolizumab treatment (p <0.05) and at the last visit (p <0.01) compared with those at the start of mepolizumab treatment. Conclusions: Treatment with mepolizumab in the super-responder group durably reduced the relapse rate.

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“…We obtained a patient-reported outcome (PRO) from the questionnaire. The maximum scores of motor and sensory evaluations are 48 and 40, respectively (3). In this PRO, a higher motor score indicates a better motor function as assessed by the patient, whereas a higher sensory score indicates a lower sensation function as assessed by the patient.…”

Section: Case Reportmentioning

confidence: 99%

Long-term Safety and Efficacy of Benralizumab for Eosinophilic Granulomatosis with Polyangiitis Complicated with Severe Neuropathy

Koga

Yoshimi²,

Harada³

et al. 2023

Intern. Med.

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The efficacy of benralizumab, as well as mepolizumab, to granulomatosis with polyangiitis (EGPA) involved with mononeuritis multiplex remains unclear. We experienced a case of EGPA presenting neuropathy with severe asthma. Muscle weakness due to neuropathy involved with gait disturbance was partly ameliorated by intravenous immunoglobulin therapy. Mepolizumab (100 mg/day) did not promote further improvement of neuropathy. However, the administration of benralizumab instead of mepolizumab improved neuropathy quickly and enabled walking alone. The efficacy of benralizumab for EGPA and its complication has been maintained for over four years. Benralizumab may be a possible treatment for EGPA presenting neuropathy with severe asthma.

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Familial Eosinophilic Granulomatosis with Polyangiitis in a Sister and Brother

Cited by 7 publications

References 18 publications

Intravenous cyclophosphamide and immunoglobulin ameliorated visual field defects in a patient with eosinophilic granulomatosis with polyangiitis

Intravenous cyclophosphamide and immunoglobulin ameliorated visual field defects in a patient with eosinophilic granulomatosis with polyangiitis

Long-term treatment by mepolizumab reduces the relapse rate in patients with eosinophilic granulomatosis with polyangiitis

Long-term Safety and Efficacy of Benralizumab for Eosinophilic Granulomatosis with Polyangiitis Complicated with Severe Neuropathy

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