{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,29]],"date-time":"2025-08-29T10:27:14Z","timestamp":1756463234763,"version":"3.44.0"},"reference-count":17,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2016,4,10]]},"abstract":"<jats:p>We present two cases of eosinophilic granulomatosis with polyangiitis occurring with \u03b1-1-antitrypsin deficiency, both PiSZ phenotype. The simultaneous occurrence of these two conditions has seldom been described in the literature, despite evidence of an association between \u03b1-1-antitrypsin deficiency and other forms of vasculitis. Both patients had pulmonary involvement and reported intermittent exacerbations of vasculitic symptoms. Both patients were managed on low-dose oral steroids and azathioprine remaining well with occasional exacerbations. It is important to consider whether there is an association between eosinophilic granulomatosis with polyangiitis and \u03b1-1-antitrypsin deficiency, as this may lead to more severe pulmonary symptoms during exacerbations. If a genetic association between the two conditions is found, clinicians should be aware of the possible need to screen for \u03b1-1-antitrypsin deficiency in appropriate patients.<\/jats:p>","DOI":"10.1136\/bcr-2015-214118","type":"journal-article","created":{"date-parts":[[2016,4,26]],"date-time":"2016-04-26T10:07:52Z","timestamp":1461665272000},"page":"bcr2015214118","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Rare case of eosinophilic granulomatosis with polyangiitis in two patients with \u03b1-1-antitrypsin deficiency (PiSZ)"],"prefix":"10.1136","volume":"2016","author":[{"given":"Jordan Maureen","family":"Moxey","sequence":"first","affiliation":[{"name":"University of Birmingham, Birmingham, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emma Victoria","family":"Low","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Heart of England NHS Foundation Trust, Birmingham, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alice Margaret","family":"Turner","sequence":"additional","affiliation":[{"name":"Department of Inflammatory Research Facility, Institute of Inflammation and Ageing, Birmingham, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2016,4,26]]},"reference":[{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.1","DOI":"10.1016\/S0049-0172(03)00083-0"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.2","DOI":"10.1002\/art.21250"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.3","DOI":"10.1111\/all.12088"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.4","DOI":"10.1038\/nrrheum.2014.104"},{"key":"2025082700094262000_2016.apr26_1.bcr2015214118.5","first-page":"868","article-title":"Chromosomal localization of the human alpha 1antitrypsin gene (PI) to 14q31-32","volume":"37","author":"Schroeder","year":"1985","journal-title":"Am J Hum Genet"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.6","DOI":"10.1164\/rccm.201108-1428CI"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.7","DOI":"10.1093\/oxfordjournals.ndt.a027308"},{"key":"2025082700094262000_2016.apr26_1.bcr2015214118.8","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1016\/0168-9525(89)90200-X","article-title":"The alpha-1-antitrypsin gene and its deficient states","volume":"5","author":"Crystal","year":"1989","journal-title":"Trends Genet"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.9","DOI":"10.1002\/art.27742"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.10","DOI":"10.1136\/ard.2011.153569"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.11","DOI":"10.1038\/ki.1993.186"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.12","DOI":"10.1111\/j.1365-2796.1994.tb00842.x"},{"key":"2025082700094262000_2016.apr26_1.bcr2015214118.13","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.jaut.2014.01.018","article-title":"Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome)","volume":"48\u201349","author":"Mouthon","year":"2014","journal-title":"J Autoimmun"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.14","DOI":"10.1016\/S0140-6736(15)60860-1"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.15","DOI":"10.1186\/1750-1172-6-14"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.16","DOI":"10.1016\/0190-9622(95)90436-0"},{"doi-asserted-by":"publisher","key":"2025082700094262000_2016.apr26_1.bcr2015214118.17","DOI":"10.1007\/s00296-013-2745-9"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2015-214118","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T07:09:55Z","timestamp":1756278595000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2015-214118"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,4,26]]},"references-count":17,"alternative-id":["10.1136\/bcr-2015-214118"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2015-214118","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2016,4,26]]}}}