{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T21:57:05Z","timestamp":1793224625460,"version":"4.5.0"},"reference-count":17,"publisher":"The Journal of Rheumatology","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2013,6]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>To date only 38 cases of childhood-onset eosinophilic granulomatosis with polyangiitis (cEGPA; formerly Churg-Strauss syndrome) have been reported. Additional patients with cEGPA could enhance the understanding of this rare and life-threatening condition. Our objectives were (1) to determine the frequency of specific organ system involvement; (2) to examine initial therapeutic regimen; and (3) to document disease and therapy-related morbidity in a contemporary cohort of patients with cEGPA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>Retrospective review of patients evaluated at the Cleveland Clinic between 2003 and 2011 who met either American College of Rheumatology or Lanham criteria for EGPA and whose age was &lt; 18 years at symptom onset.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>Nine patients (8 female; 7 white) were identified. Median age at onset of rhinitis\/asthma symptom was 13 years and median age at diagnosis of cEGPA was 15 years. All patients demonstrated eosinophilia, upper airway disease (allergic rhinitis, chronic sinusitis, and\/or nasal polyps), and pulmonary involvement. Other frequently involved organ systems included musculoskeletal (67%), gastrointestinal (67%), cutaneous (67%), neurologic (56%), and cardiac (44%). Antineutrophil cytoplasmic antibody (ANCA) serologies were negative in all patients. The medications used most frequently for initial therapy included oral (44%) or intravenous corticosteroids (56%) and azathioprine (67%). Disease or therapeutic complications occurred in half of the cohort and included heart failure, stroke, and sequela from longterm, high-dose steroids.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>Eosinophilia, in combination with upper airway, pulmonary, musculoskeletal, neurologic, and cardiac manifestations, is frequently observed in cEGPA. ANCA titers are often negative. Steroids are the mainstay of initial therapy but steroid-related side effects occur regularly.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.120808","type":"journal-article","created":{"date-parts":[[2013,5,2]],"date-time":"2013-05-02T08:27:39Z","timestamp":1367483259000},"page":"929-935","source":"Crossref","is-referenced-by-count":68,"title":["Childhood-onset Eosinophilic Granulomatosis with Polyangiitis (formerly Churg-Strauss Syndrome): A Contemporary Single-center Cohort"],"prefix":"10.3899","volume":"40","author":[{"given":"Samantha","family":"Gendelman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Zeft","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven J.","family":"Spalding","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2262","published-online":{"date-parts":[[2013,5,1]]},"reference":[{"key":"2018112909313785000_40.6.929.1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1099-1557(199905\/06)8:3<179::AID-PDS414>3.0.CO;2-K"},{"key":"2018112909313785000_40.6.929.2","doi-asserted-by":"publisher","DOI":"10.1097\/00002281-200301000-00003"},{"key":"2018112909313785000_40.6.929.3","doi-asserted-by":"publisher","DOI":"10.1002\/art.20077"},{"key":"2018112909313785000_40.6.929.4","first-page":"277","article-title":"Allergic granulomatosis, allergic angiitis, and periarteritis nodosa","volume":"27","author":"Churg","year":"1951","journal-title":"Am J Pathol"},{"key":"2018112909313785000_40.6.929.5","doi-asserted-by":"publisher","DOI":"10.1002\/art.21250"},{"key":"2018112909313785000_40.6.929.6","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-143-9-200511010-00006"},{"key":"2018112909313785000_40.6.929.7","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780330806"},{"key":"2018112909313785000_40.6.929.8","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1097\/00005792-198403000-00001","article-title":"Systemic vasculitis with asthma and eosinophilia: A clinical approach to the Churg-Strauss syndrome","volume":"63","author":"Lanham","year":"1984","journal-title":"Medicine"},{"key":"2018112909313785000_40.6.929.9","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2008.05.004"},{"key":"2018112909313785000_40.6.929.10","doi-asserted-by":"crossref","first-page":"977","DOI":"10.6061\/clinics\/2012(08)22","article-title":"A possible case of Churg-Strauss syndrome in a 9-year-old child","volume":"67","author":"Liu","year":"2012","journal-title":"Clinics (Sao Paulo)"},{"key":"2018112909313785000_40.6.929.11","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1080\/02724936.1995.11747795","article-title":"Churg Strauss syndrome in childhood","volume":"15","author":"Mpofu","year":"1995","journal-title":"Ann Trop Paediatr"},{"key":"2018112909313785000_40.6.929.12","doi-asserted-by":"crossref","unstructured":"Mutsaers ER Witteveen R van den Bosch-Ruis W Kuijpers TW van Houten MA van den Berg JM . 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