Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome

Kim, Sophia; Marigowda, Gautham; Oren, Eyal; Israel, Elliot; Wechsler, Michael E.

doi:10.1016/j.jaci.2010.03.028

Cited by 256 publications

(141 citation statements)

References 26 publications

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“…In our fourth case, relapse did not occur after the initial treatment. In the recent years, it was reported that cyclophosphamide, intravenous immunoglobulin, rituximab, and mepolizumab, which is an anti-IL-5 antibody, can also be used in EGPA patients where corticosteroid is not sufficient [37][38][39].…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic Lung Disease: Accompanied with 12 Cases

et al. 2015

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OBJECTIVES:Eosinophilic lung diseases are a rare group of heterogeneous diseases characterized by the increase of the eosinophil ratio in airways and lung parenchyma. In our clinic, patients diagnosed with eosinophilic lung disease were evaluated with their clinical features and prognoses. MATERIAL AND METHODS:In our clinic, 12 cases that were diagnosed and followed up for eosinophilic lung disease [eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) (n=4), chronic eosinophilic pneumonia (CEP) (n=7), and simple pulmonary eosinophilia (Löffler's syndrome) (n=1)] were retrospectively evaluated. RESULTS:Of the 12 cases, 8 were females, and the average age was 43 (28-72) years. All cases were undergoing bronchodilator therapy with asthma diagnosis (2 months-40 years). Additionally, 4 of the cases had sinusitis, and 1 had allergic rhinitis. The most common complaints of the patients were difficulty in breathing and coughing, and the duration of complaints was a median of 2 months. Peripheral eosinophilia and total IgE elevation were present during the admission of all cases; additionally, leucocyte elevation was recorded in 10 of them, anemia in 4 of them, and thrombocytosis in 4 of them. Moreover, 43% of the recorded DLCO values were lower than normal. Of the 10 cases that underwent bronchoalveolar lavage (BAL), the eosinophil ratio was above 25% in 7 subjects. Of the 8 cases that underwent transbronchial biopsy, eosinophil-involving infiltration was detected in 6 subjects. Additional findings in cases diagnosed with EGPA were nasal polyposis (n=1), sinusitis (n=2), polyneuropathy (n=1), cardiac involvement (n=2), and skin involvement in biopsy (n=1). Spontaneous recovery was observed in the patient diagnosed with simple pulmonary eosinophilia during the follow-up that was performed based on the history and laboratory and BAL results of the patient. Prednisolone treatment was started for all cases, except for simple pulmonary eosinophilia, and their controls were performed. Relapse was observed in eight cases (EGPA: 4, CEP: 4); during the relapse treatment of one case diagnosed with EGPA, exitus occurred. One case rejected treatment despite the presence of peripheral eosinophilia, and the other cases are being followed-up without medication. CONCLUSION:Given that the clinical pictures in pulmonary eosinophilia syndromes are on a wide spectrum, a specific diagnosis is important. Progression may differ in each patient, and a close follow-up is necessary during and after the treatment. 24.12.2014 KEYWORDS:

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Section: Discussionmentioning

confidence: 99%

Eosinophilic Lung Disease: Accompanied with 12 Cases

et al. 2015

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“…Moreover, rituximab can be also used as maintenance therapy but the level of evidence is low (grade 3C). Finally, studies including biological agents such as etanercept, adalimumab, abatacept, altemtuzumab, and mepolizumab either did not include patients with neurological involvement, or the results were inconclusive [137][138][139].…”

Section: Antineutrophil Cytoplasmic Antibody-positive Vasculitidesmentioning

confidence: 99%

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

et al. 2016

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Neurological involvement is relatively common in the majority of systemic autoimmune diseases and may lead to severe morbidity and mortality, if not promptly treated. Treatment options vary greatly, depending on the underlying systemic pathophysiology and the associated neurological symptoms. Selecting the appropriate therapeutic scheme is further complicated by the lack of definite therapeutic guidelines, the necessity to differentiate primary neurological syndromes from those related to the underlying systemic disease, and to sort out adverse neurological manifestations caused by immunosuppressants or the biological agents used to treat the primary disease. Immunotherapy is a sine qua non for treating most, if not all, neurological conditions presenting in the context of systemic autoimmunity. Specific agents include classical immune modulators such as corticosteroids, cyclophosphamide, intravenous immunoglobulin, and plasma exchange, as well as numerous biological therapies, for example antitumor necrosis factor agents and monoclonal antibodies that target various immune pathways such as B cells, cytokines, and co-stimulatory molecules. However, experience regarding the use of these agents in neurological complications of systemic diseases is mainly empirical or based on small uncontrolled studies and case series. The aim of this review is to present the state-of-the-art therapies applied in various neurological manifestations encountered in the context of systemic autoimmune diseases; evaluate all treatment options on the basis of existing guidelines; and compliment these data with our personal experience derived from a large number of patients.

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“…Mepolizumab, a humanized monoclonal antibody against IL-5, has also generated promising results in eosinophilic asthma [29][30][31][32] and EGPA [33,34]. Two pilot studies with mepolizumab in EGPA have reported a corticosteroid sparing effect and remission maintenance in the absence of further conventional immunosuppressants [33,34].…”

Section: Editorialmentioning

confidence: 99%

“…Two pilot studies with mepolizumab in EGPA have reported a corticosteroid sparing effect and remission maintenance in the absence of further conventional immunosuppressants [33,34]. A large randomized, double-blind, phase III study is currently investigating the efficacy and safety of mepolizumab compared with placebo in subjects with relapsing or refractory EGPA receiving standard of care therapy including background corticosteroid therapy with or without immunosuppressive therapy (MIRRA, ClinicalTrials.gov Identifier: NCT02020889).…”

Section: Editorialmentioning

confidence: 99%

Immunotherapy in Eosinophilic Granulomatosis with Polyangiitis: A New Step Forward?

Puéchal¹

2016

J Vasc

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Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome

Cited by 256 publications

References 26 publications

Eosinophilic Lung Disease: Accompanied with 12 Cases

Eosinophilic Lung Disease: Accompanied with 12 Cases

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

Immunotherapy in Eosinophilic Granulomatosis with Polyangiitis: A New Step Forward?

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