Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease

Berti, Alvise; Boukhlal, Sara; Groh, Matthieu; Cornec, Divi

doi:10.1080/1744666x.2019.1697678

Cited by 24 publications

(19 citation statements)

References 82 publications

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“…Clinical and/or pathologic features of vasculitis, such as glomerulonephritis or peripheral neuropathy, can be observed in both patients with ANCA-negative EGPA and those with ANCA-positive EGPA. Moreover, some disease manifestations including peripheral neuropathy or cardiomyopathy could be due to both vasculitis and eosinophilic infiltration (46). Given the primacy of the association of genetic polymorphisms with ANCA serotype, future disease classifications based on serotype will have an influence on the subclassification of EGPA, with the possibility that PR3-ANCA and MPO-ANCA-positive EGPA will become eosinophilic variants of PR3-ANCA and MPO-ANCA-positive AAV.…”

Section: Clinical Value Of Anca In Egpamentioning

confidence: 99%

International Consensus on Antineutrophil Cytoplasm Antibodies Testing in Eosinophilic Granulomatosis with Polyangiitis

Moiseev

Bossuyt

Arimura

et al. 2020

Am J Respir Crit Care Med

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Section: Clinical Value Of Anca In Egpamentioning

confidence: 99%

International Consensus on Antineutrophil Cytoplasm Antibodies Testing in Eosinophilic Granulomatosis with Polyangiitis

Moiseev

Bossuyt

Arimura

et al. 2020

Am J Respir Crit Care Med

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“…In addition, 30% of subjects suffer from a precursive chronic eosinophilic pneumonia. A recent report by Berti et al showed that uncontrolled asthmatic symptoms are associated with baseline pulmonary, ear, nose, and throat manifestations, but not with vasculitic symptoms among asthmatic patients with EGPA [17]. An atopic factor was also related to a prognosis among asthmatic patients with EGPA [18].…”

Section: Damage To Each Organ Due To Egpamentioning

confidence: 94%

“…EGPA usually develops in adulthood and subjects suffer from severe asthmatic and eosinophilic sinusitis symptoms that arise from prominent eosinophilic hyperplasia [16,17]. The severe type of eosinophilic sinusitis is often associated with nasal polyps, which cause decreased olfaction, which impairs quality of life (QOL).…”

Section: Damage To Each Organ Due To Egpamentioning

confidence: 99%

Role of Cytokines in EGPA and the Possibility of Treatment with an Anti-IL-5 Antibody

Isozaki

Homma

Sagara

et al. 2020

JCM

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Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of systemic vasculitis with eosinophilia in the peripheral blood, which is preceded by bronchial asthma or allergic disease. EGPA is pathologically characterized by microangiopathy granulomatosis vasculitis. Vasculitis can be exacerbated and cause central nervous system and cardiovascular disorders and gastrointestinal perforation. Histological examination reveals eosinophil infiltration and granulomas in lesions in areas such as the lung, nervous system, and skin. Laboratory tests show inflammatory findings such as C-reactive protein (CRP) elevation, increased eosinophils, elevated serum IgE, and elevated myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA). MPO-ANCA is positive in approximately 40–70% of cases of this disease. EGPA is a necrotizing vasculitis that affects small- and medium-sized blood vessels; however, it differs from other types of ANCA-related vasculitis (such as microscopic polyangiitis and granulomatosis) because it is preceded by bronchial asthma and eosinophilia in the blood and tissues. Treatment with immunosuppressive agents such as steroids or cyclophosphamide depends on the Five Factor Score, which predicts the prognosis and severity of the condition. If the effect of appropriate treatment with steroids is insufficient, the anti-interleukin-5 antibody mepolizumab can be administered. The combination of mepolizumab with standard treatment leads to a significantly longer duration of remission, a higher proportion of patients who achieve sustained remission, and less steroid use than with a placebo.

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“…отрицательный результат серологического исследования не позволяет сделать вывод об отсутствии васкулита. С другой стороны, некоторые проявления болезни, например, поражение нервной системы или сердца, могут быть следствием как васкулита, так и эозинофильной инфильтрации тканей [11].…”

Section: критерии классификации эгпаunclassified

Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis

Novikov¹,

Bulanov²,

Zykova³

et al. 2020

CPT

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Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome; EGPA) is an ANCA-associated vasculitis characterized by the presence of eosinophilic asthma/rhinosinusitis and the disease of peripheral nervous system, kidneys, skin, heart and other organs. ANCA can be detected in a one third of EGPA patients. Initially, patients with EGPA are usually treated with glucocorticoids ± immunosuppressive agents. Mepolizumab, a monoclonal antibody against interleukin (IL)-5, can be used in patients with refractory or recurrent EGPA who do not respond to standard therapy. IL-5 is the main cytokine that regulates differentiation, activation, survival, and maturation of eosinophils. Efficacy and favorable safety profile of mepolizumab were established in the double-blind, placebo-controlled, clinical trial in 136 EGPA patients. Administration of mepolizumab compared to placebo resulted in significant improvement in remission and relapses rates and allowed for reduced glucocorticoid use.

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Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease

Cited by 24 publications

References 82 publications

International Consensus on Antineutrophil Cytoplasm Antibodies Testing in Eosinophilic Granulomatosis with Polyangiitis

International Consensus on Antineutrophil Cytoplasm Antibodies Testing in Eosinophilic Granulomatosis with Polyangiitis

Role of Cytokines in EGPA and the Possibility of Treatment with an Anti-IL-5 Antibody

Mepolizumab for the treatment of eosinophilic granulomatosis with polyangiitis

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