Targeting Interleukin-5 in Refractory and Relapsing Churg–Strauss Syndrome

Moosig, Frank; Gross, Wolfgang L.; Herrmann, Kristina; Bremer, Jan Phillip; Hellmich, Bernhard

doi:10.7326/0003-4819-155-5-201109060-00026

Cited by 192 publications

(101 citation statements)

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“…Eight of the 10 recruited patients reached remission and were able to reduce the dose of glucocorticoids to below 7.5 mg/d. No patient relapsed during treatment, but clinical manifestations reappeared after the withdrawal of mepolizumab [98].…”

Section: Biological Therapies (Monoclonal Antibodies)mentioning

confidence: 99%

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

et al. 2015

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Eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA) is a systemic small-to-medium-sized vasculitis associated with asthma and eosinophilia. Histologically EGPA presents tissue eosinophilia, necrotizing vasculitis, and granulomatous inflammation with eosinophil tissue infiltration. EGPA commonly involves the upper airway and lung parenchyma, peripheral neuropathy, cardiac disorders, and skin lesions. The anti-neutrophil cytoplasmic antibodies (ANCA) are positive in 40% of cases, especially in those patients with clinical signs of vasculitis. The pathogenesis of EGPA is multifactorial. The disease can be triggered by exposure to a variety of allergens and drugs, but a genetic background has also been described, particularly an association with HLA-DRB4. Th2 response is of special importance in the upregulation of different interleukins such as IL-4, IL-13, and IL-5. Th1 and Th17 responses are also of significance. Activated eosinophils have a prolonged survival and probably cause tissue damage by releasing eosinophil granule proteins, while their tissue recruitment can be regulated by chemokines such as eotaxin-3 and CCL17. Humoral immunity is also OPEN ACCESSSinusitis 2016, 1 25 abnormally regulated, as demonstrated by excessive responses of IgG4 and IgE. EGPA has a good respond to glucocorticoids, although the combination of glucocorticoids and immunosuppressants (e.g., cyclophosphamide, azathioprine) is needed in most of cases. Newer treatment options include anti-IL-5 antibodies (mepolizumab), whose efficacy has been described in clinical trials, and anti-CD-20, a B cell-depleting agent (rituximab), reported in several case series.

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Section: Biological Therapies (Monoclonal Antibodies)mentioning

confidence: 99%

Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

et al. 2015

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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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“…Azathioprine or methotrexate is also used. Biologic therapies are currently under investigation [7][8][9][10]. In CSS, the most commonly involved organ is the lung, followed by the skin.…”

Section: Introductionmentioning

confidence: 99%