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Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is described in the packet's definition as a systemic vasculitis of small to medium vessels, characterized by asthma, transient pulmonary infiltrates, and hypereosinophilia. Orphanet is among the cited sources for this condition. The packet classifies its prevalence as uncommon, at 1-9 in 100,000. Synonyms recorded for the condition include CSS and Churg-Strauss vasculitis.
The packet does not list individual phenotype annotations for this condition. Its definition names asthma, transient pulmonary infiltrates, and hypereosinophilia as characteristic features of the disease. Age of onset and the order or severity of features are not certified in this packet.
The packet lists no associated genes and no inheritance pattern for this condition, and it does not certify a specific underlying cause or mechanism. The definition describes the condition as a systemic vasculitis, meaning inflammation of blood vessels.
Diagnostic methods and classification criteria are not certified in this packet. The characteristic features named in the definition are asthma, transient pulmonary infiltrates, and hypereosinophilia.
FDA records in the packet list two products with active market status for this condition: mepolizumab (marketed as Nucala, a BLA approved on 2019-06-06) and benralizumab (marketed as Fasenra, a BLA approved on 2017-11-14). Orphan drug records in the packet also show an approved status for benralizumab and mepolizumab, and for avacopan (Tavneos) and rituximab (Rituxan), the latter two with indications described as ANCA-associated vasculitides that include Churg-Strauss syndrome. A selective Janus kinase 1 inhibitor from NS Pharma carries an orphan designation for this condition; a designation is not an approval. Other management approaches are not certified in this packet.
20 trials found
Prognosis, natural history, and long-term outcomes are not certified in this packet.
ClinicalTrials.gov records in the packet show numerous trials for this condition, with studies currently recruiting or active but not recruiting. Listed studies include phase 3 trials comparing benralizumab with mepolizumab (sponsored by AstraZeneca) and depemokimab with mepolizumab (sponsored by GlaxoSmithKline), phase 2 trials of NS-229, tezepelumab, and SHR-1703, and a recruiting study titled Benralizumab Effectiveness in EGPA from the European EGPA Study Group. Several recruiting records are registries or repositories for systemic vasculitis, including the Vasculitis Pregnancy Registry and the VCRC Tissue Repository. A trial title indicates the agent under study and does not establish an intervention's effect. ClinicalTrials.gov provides a searchable listing of studies for this condition.
Data assembled from 5 of 12 sources · Last updated Nov 2, 2026, 9:32 PM UTC
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AI-curated news mentioning eosinophilic granulomatosis with polyangiitis
Updated Oct 19, 2026
A recent study explores isolated bilateral periocular lupus miliaris disseminatus faciei, highlighting its clinicopathological similarities to granulomatosis with polyangiitis. This research may aid in differentiating these conditions in clinical practice.
Recent clinical experience highlights the effectiveness of anti-interleukin-5 monoclonal antibodies in treating eosinophilic granulomatosis with polyangiitis. This research contributes to understanding targeted therapies for this rare autoimmune condition.
A recent study investigates the use of serial ANCA testing to predict relapse risk in patients with granulomatosis with polyangiitis. The findings could enhance monitoring strategies for this rare autoimmune disease.