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      Switching from omalizumab to mepolizumab therapy improved extra‐pulmonary abdominal and cutaneous vasculitis symptoms in a patient with eosinophilic granulomatosis with polyangiitis

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          Abstract

          Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis of small‐to‐medium‐sized vessels. Both eosinophilic infiltration and vasculitis are thought to contribute to multi‐organ damage. Some biologics have been used to reduce the required dose of corticosteroids in EGPA, but no single agent can ensure a complete control of this disease. Here, we describe a patient with anti‐neutrophil cytoplasmic antibodies‐negative relapsing EGPA whose asthma control was improved by omalizumab, but she continued to develop flares of abdominal and cutaneous vasculitis symptoms. After switching to mepolizumab therapy, her blood hypereosinophilia and extra‐pulmonary symptoms were significantly improved. Moreover, the dose of daily maintenance corticosteroid could be tapered off. The experience from our case suggests that biologics targeting interleukin‐5 may be more effective than omalizumab in the management of extra‐thoracic manifestations in EGPA.

          Abstract

          We report the clinical benefits after switching from omalizumab to mepolizumab in an anti‐neutrophil cytoplasmic antibodies‐negative eosinophilic granulomatosis with polyangiitis patient on her abdominal and cutaneous manifestations.

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          Most cited references30

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          2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.

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            Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis.

            Eosinophilic granulomatosis with polyangiitis is an eosinophilic vasculitis. Mepolizumab, an anti-interleukin-5 monoclonal antibody, reduces blood eosinophil counts and may have value in the treatment of eosinophilic granulomatosis with polyangiitis.
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              Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort.

              Earlier studies of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), with limited patient numbers and followup durations, demonstrated that clinical presentation at diagnosis, but not outcome, differed according to antineutrophil cytoplasmic antibody (ANCA) status. This study was undertaken to describe the main characteristics of a larger patient cohort and their long-term outcomes. A retrospective study of EGPA patients in the French Vasculitis Study Group cohort who satisfied the American College of Rheumatology criteria and/or Chapel Hill definitions was conducted. Patient characteristics and outcomes were compared according to ANCA status and year of diagnosis. We identified 383 patients diagnosed between 1957 and June 2009 (128 [33.4%] before 1997 or earlier) and followed up for a mean±SD of 66.8±62.5 months. At diagnosis, their mean±SD age was 50.3±15.7 years, and 91.1% had asthma (duration 9.3±10.8 years). Main manifestations included peripheral neuropathy (51.4%); ear, nose, and throat (ENT) signs (48.0%); skin lesions (39.7%); lung infiltrates (38.6%); and cardiomyopathy (16.4%). Among the 348 patients tested at diagnosis for ANCA, the 108 ANCA-positive patients (31.0%) had significantly more frequent ENT manifestations, peripheral neuropathy, and/or renal involvement, but less frequent cardiac manifestations, than the ANCA-negative patients. Vasculitis relapses occurred in 35.2% of the ANCA-positive versus 22.5% of the ANCA-negative patients (P=0.01), and 5.6% versus 12.5%, respectively, died (P<0.05). The 5-year relapse-free survival rate was 58.1% (95% confidence interval [95% CI] 45.6-68.6) for ANCA-positive and 67.8% (95% CI 59.8-74.5) for ANCA-negative patients (P=0.35). Multivariable analysis identified cardiomyopathy, older age, and diagnosis during or prior to 1996 as independent risk factors for death and lower eosinophil count at diagnosis as predictive of relapse. The characteristics and long-term outcomes of EGPA patients differ according to their ANCA status. Although EGPA relapses remain frequent, mortality has declined, at least since 1996. Copyright © 2013 by the American College of Rheumatology.
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                Author and article information

                Contributors
                kph712@ntuh.gov.tw
                Journal
                Respirol Case Rep
                Respirol Case Rep
                10.1002/(ISSN)2051-3380
                RCR2
                Respirology Case Reports
                John Wiley & Sons, Ltd (Chichester, UK )
                2051-3380
                29 November 2021
                January 2022
                : 10
                : 1 ( doiID: 10.1002/rcr2.v10.1 )
                : e0878
                Affiliations
                [ 1 ] Department of Internal Medicine National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan
                Author notes
                [*] [* ] Correspondence

                Ping‐Hung Kuo, Department of Internal Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, No. 7, Zhongshan S. Rd., Zhongzheng Dist., Taipei 100, Taiwan.

                Email: kph712@ 123456ntuh.gov.tw

                Author information
                https://orcid.org/0000-0003-1984-2374
                https://orcid.org/0000-0003-3756-3395
                Article
                RCR2878
                10.1002/rcr2.878
                8629745
                1a529bf8-7ad9-4a39-a213-5cefd5a87a12
                © 2021 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                : 14 September 2021
                : 10 June 2021
                : 08 November 2021
                Page count
                Figures: 3, Tables: 1, Pages: 5, Words: 3191
                Categories
                Case Report
                Case Reports
                Custom metadata
                2.0
                January 2022
                Converter:WILEY_ML3GV2_TO_JATSPMC version:6.0.9 mode:remove_FC converted:29.11.2021

                eosinophilic granulomatosis with polyangiitis,extra‐pulmonary,mepolizumab,omalizumab,switching

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