Longterm Outcomes of 188 Japanese Patients with Eosinophilic Granulomatosis with Polyangiitis

Saku, Aiko; Furuta, Shunsuke; Hiraguri, Masaki; Ikeda, Kei; Kobayashi, Y.; Kagami, Shin-ichiro; Kurasawa, Kazuhiro; Matsumura, Ryutaro; Nakagomi, Daiki; Sugiyama, Takao; Umibe, Takeshi; Watanabe, Norihiko; Nakajima, Hiroshi

doi:10.3899/jrheum.171352

Cited by 45 publications

(26 citation statements)

References 22 publications

(44 reference statements)

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“…CNS involvement in the current study was found in less than one-fifth of EGPA patients, suggesting that it was relatively rare compared to peripheral neuropathy in EGPA, which occurred in 46.4% of patients [11]. In studies from other countries, CNS lesions occurred in 5-14% of EGPA patients [7,[12][13][14][15][16], which was lower than that in our cohort. The reason may be that most of the patients admitted to our hospital were referred from the local hospitals with severe conditions.…”

Section: Discussioncontrasting

confidence: 79%

Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China

Liu

Guo

Fan

et al. 2021

Orphanet J Rare Dis

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Background Central nervous system (CNS) involvement is extremely rare in eosinophilic granulomatosis with polyangiitis (EGPA), but is associated with a poor prognosis in the five-factor score. This study aims to elucidate the clinical features and independently associated factors of EGPA with CNS involvement. Results CNS involvement was observed in 17.3% (19/110) of EGPA patients from Peking Union Medical College Hospital between 2007 and 2019. We retrospectively reviewed their clinical data and analyzed the independently associated factors. Their mean age was 51.7 ± 11.56 years with no male/female predominance. Ischemic lesions were the most common manifestations, accounting for 63.2% of the 19 cases, followed by posterior reversible encephalopathy syndrome (36.8%), spinal cord involvement (15.8%), medulla oblongata involvement (15.8%), and intracranial hemorrhages (15.8%). Compared to the control group, patients with CNS involvement were of older age (51.7 ± 11.56 vs. 43.7 ± 13.78 years, p = 0.019) and had a higher ratio in the digestive tract involvement (52.6% vs. 28.6%, p = 0.042). Further multivariate analysis revealed that age, disease duration, and fever were the potential independent risk factors for CNS involvement of EGPA. Glucocorticoids combined with cyclophosphamide were the strategic therapy (94.7%). Intrathecal injections of dexamethasone and methotrexate were administered to 21.1% of the patients. Although seven patients relapsed during glucocorticoid reduction, seventeen patients finally achieved clinical remission. One patient died of acute intracerebral hemorrhage within one month, and another died of gastrointestinal perforation. Outcomes and cumulative survival show no significant differences between the two groups. Conclusions CNS involvement is uncommon in EGPA with various manifestations, and ischemic lesions are the most frequent. Age, disease duration, and fever are independent factors associated with CNS involvement in EGPA. The therapy of glucocorticoids combined with cyclophosphamide and intrathecal injections yields favorable responses. Acute intracranial hemorrhage and gastrointestinal perforation may be the principal causes of death.

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Section: Discussioncontrasting

confidence: 79%

Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China

Liu

Guo

Fan

et al. 2021

Orphanet J Rare Dis

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“…Blood eosinophil has been previously related to disease burden and outcomes of CRS, and asthma and eosinophilic granulomatosis with polyangiitis. [9][10][11][12][27][28][29] Despite studies indicating distinct endotypes in CRS patients, 30,31 our study aimed to understand the associations between uncontrolled disease and inflammatory patterns. We analyzed potential predictors in Chinese CR-SwNP patients and subgroups classified based on the site of eosinophilia.…”

Section: Discussionmentioning

confidence: 99%

Inflammatory features and predictors for postsurgical outcomes in patients with nasal polyps stratified by local and systemic eosinophilia

Liao

Guo

et al. 2020

Int Forum Allergy Rhinol

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BackgroundIn this study we aimed to identify inflammatory patterns and predictors associated with clinical outcomes in chronic rhinosinusitis with nasal polyps CRSwNP patients with different blood and tissue eosinophilia Methods A total of CRSwNP patients were enrolled and the expression of biomarkers together with eosinophil and neutrophil counts in nasal polyps were analyzed in a subset of patients Patients were stratified on the basis of blood ≥ × /L and tissue > eosinophilia Logistic regression models were applied to identify predictors of uncontrolled disease at least year a er surgery Uncontrolled disease was defined according to the European Position Paper on Rhinosinusitis and Nasal Polyps Results Among patients showed inconsistent blood and tissue eosinophilia In CRSwNP patients subjects with concomitant blood and tissue eosinophilia group showed marked mucosal type inflammation characterized by high levels of interleukin IL -IL-and eotaxin-whereas subjects with normal blood and tissue eosinophil levels group demonstrated significant local neutrophilic inflammation with high expression of granulocyte colony-stimulating factor and subjects with selec-tive tissue eosinophilia group showed intermediate and mixed eosinophilic and neutrophilic inflammation Subjects with isolated blood eosinophilia group showed low expression of vascular endothelial growth factor and IL-Asthma prior sinus surgery and blood eosinophilia were the top predictors for postsurgical uncontrolled disease For subgroup analysis sex in group asthma in group tissue IL-and immunoglobulin E in group and prior sinus surgery in group were the strongest predictors of uncontrolled disease respectively Conclusion Different blood and tissue eosinophilia revealed distinct tissue inflammatory patterns in CRSwNP patients © 2020 ARS-AAOA, LLC.

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“…Despite the standard of care, EGPA often relapses during CS dose reduction, and hence, CS dose reduction is often challenging. [3][4][5] In recent years, mepolizumab (MPZ), an anti-interleukin-5 antibody, has been reported to extend the remission period of EGPA and reduce CS dose. 6 Although MPZ was listed in the National Health Insurance drug price list for treating EGPA in Japan in 2018, its effectiveness and safety have not been adequately evaluated in real-world clinical practice.…”

Section: Introductionmentioning

confidence: 99%

“…In the with-MPZ, VDI scores were 4.0 [3.0−5.5] at month 1, 4.0 [3.0−5.5] at month 3, 4.0 [3.0−5.5] at month 6, and 4.0 [3.0−5.5] at month 12, showing no signi cant changes (Figure 1C). The increase in the VDI score during the 1-year period was 0 [0−0.8] in the without-MPZ and 0 [0−0] in the with-MPZ, showing no signi cant difference between the two groups (Figure 2B).In the without-MPZ, eosinophil counts were 280 4. [63.2−426.8]/μL at month 1, 217.8 [93.3−1354.2]/μL at month 3, and 293.9 [39.1−880.7]/μL at month 6, showing no signi cant changes compared with those at month 0.…”

mentioning

confidence: 97%

Effectiveness and Safety of Mepolizumab in Combination with Corticosteroids in Patients with Eosinophilic Granulomatosis with Polyangiitis

Ueno

Miyagawa

Nakano

et al. 2020

Preprint

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Objectives: Mepolizumab (MPZ), an anti-interleukin-5 antibody, is effective for treating eosinophilic granulomatosis with polyangiitis (EGPA). However, its effectiveness has not been adequately evaluated in real-world clinical practice. In this study, we assessed the effectiveness and safety of 300 mg MPZ for relapsing/refractory EGPA resistant to corticosteroids (CS) for 1 year in real-world settings. Methods: We administered MPZ (300 mg) to 16 patients with relapsing/refractory EGPA resistant to CS (with-MPZ). We also retrospectively collected data from the same patients for 12 months before administering MPZ (without-MPZ). The primary endpoint was the 12-month remission rate after MPZ administration, and the secondary endpoints were the Birmingham vasculitis activity score (BVAS), vasculitis damage index (VDI), eosinophil count, changes in concomitant CS doses/concomitant immunosuppressant use, MPZ retention rate, and incidence of adverse events. The clinical course was compared between Without-MPZ and With MPZ.Results: The 12-month remission rate after initiating MPZ was 75%. No change was observed in BVAS, eosinophil count, or concomitant CS dose in the without-MPZ, whereas all these parameters were significantly decreased in the with-MPZ. The number of patients on concomitant immunosuppressants also decreased in the with-MPZ. VDI did not increase in both groups. The MPZ retention rate was 100%, and only three patients (18.8%) had infections.Conclusion: This study demonstrated that MPZ is effective and safe for EGPA, furthermore, compared to Without-MPZ, MPZ improves disease activity and possesses a higher remission rate and CS sparing effect.

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Longterm Outcomes of 188 Japanese Patients with Eosinophilic Granulomatosis with Polyangiitis

Cited by 45 publications

References 22 publications

Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China

Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China

Inflammatory features and predictors for postsurgical outcomes in patients with nasal polyps stratified by local and systemic eosinophilia

Effectiveness and Safety of Mepolizumab in Combination with Corticosteroids in Patients with Eosinophilic Granulomatosis with Polyangiitis

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