{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,14]],"date-time":"2026-01-14T04:19:40Z","timestamp":1768364380878,"version":"3.49.0"},"reference-count":30,"publisher":"American Academy of Pediatrics (AAP)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,8,1]]},"abstract":"<jats:p>Beh\u00e7et disease (BD) is a multisystemic autoinflammatory disorder characterized by recurrent mucocutaneous, ocular, musculoskeletal, gastrointestinal, central nervous system, and vascular manifestations. Pulmonary arterial involvement (PAI) of BD is probably the most severe form of vasculitis, at least in children. PAI has a high mortality, morbidity, and recurrence rate. There are limited data regarding treatment and outcomes of pediatric patients with BD with PAI. Herein, we report 2 pediatric patients with BD presented with hemoptysis and support our data with a systematic review. These patients were given immunosuppressive therapy, which covered pulse methylprednisolone followed by oral prednisolone, intravenous cyclophosphamide every 3 weeks for a total of 6 cycles, and interferon-\u03b12a concomitantly. These are the first reported cases in the literature successfully treated with this treatment modality in a complication with 50% mortality. These patients have been followed up for a period of at least 4 years without any vascular recurrence. Pediatricians should be aware that patients with BD may not present with full diagnostic criteria. They should consider BD in a child with PAI to avoid diagnostic delay and start life-saving accurate immunosuppressive treatment.<\/jats:p>","DOI":"10.1542\/peds.2019-0162","type":"journal-article","created":{"date-parts":[[2019,7,18]],"date-time":"2019-07-18T12:29:13Z","timestamp":1563452953000},"source":"Crossref","is-referenced-by-count":9,"title":["The Challenge of Treating Pulmonary Vasculitis in Beh\u00e7et Disease: Two Pediatric Cases"],"prefix":"10.1542","volume":"144","author":[{"given":"Selcan","family":"Demir","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erdal","family":"Sag","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ummusen","family":"Kaya Akca","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tuncay","family":"Hazirolan","sequence":"additional","affiliation":[{"name":"Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yelda","family":"Bilginer","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seza","family":"Ozen","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey; and"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"417","published-online":{"date-parts":[[2019,8,1]]},"reference":[{"key":"2021121022481954700_B1","first-page":"1152","article-title":"\u00dcber rezidivierende, apht\u00f6se, durch ein Virus verursachte Geschw\u00fcre am Mund, am Auge und an den Genitalien.","volume":"105","author":"Behcet","year":"1937","journal-title":"Dermatol Wochenschr"},{"issue":"1","key":"2021121022481954700_B2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1002\/art.37715","article-title":"2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.","volume":"65","author":"Jennette","year":"2013","journal-title":"Arthritis Rheum"},{"issue":"2","key":"2021121022481954700_B3","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1016\/j.berh.2016.08.002","article-title":"Beh\u00e7et\u2019s disease: how to diagnose and treat vascular involvement.","volume":"30","author":"Seyahi","year":"2016","journal-title":"Best Pract Res Clin Rheumatol"},{"issue":"11","key":"2021121022481954700_B4","doi-asserted-by":"crossref","first-page":"2018","DOI":"10.1093\/rheumatology\/keu233","article-title":"Vascular involvement in Beh\u00e7et\u2019s syndrome: a retrospective analysis of associations and the time course.","volume":"53","author":"Tascilar","year":"2014","journal-title":"Rheumatology (Oxford)"},{"issue":"4","key":"2021121022481954700_B5","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1007\/s00431-009-1040-y","article-title":"Beh\u00e7et disease: treatment of vascular involvement in children.","volume":"169","author":"Ozen","year":"2010","journal-title":"Eur J Pediatr"},{"issue":"5","key":"2021121022481954700_B6","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1016\/S1054-8807(02)00117-5","article-title":"Fatal hemoptysis from Behcet\u2019s disease in a child.","volume":"11","author":"Cohle","year":"2002","journal-title":"Cardiovasc Pathol"},{"issue":"5","key":"2021121022481954700_B7","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1007\/s00296-008-0730-5","article-title":"Intracardiac thrombus and pulmonary aneurysms in an adolescent with Beh\u00e7et disease.","volume":"29","author":"Vivante","year":"2009","journal-title":"Rheumatol Int"},{"issue":"6","key":"2021121022481954700_B8","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1007\/s00296-010-1363-z","article-title":"Pattern and outcome of vascular involvement of Omani patients with Behcet\u2019s disease.","volume":"31","author":"Alkaabi","year":"2011","journal-title":"Rheumatol Int"},{"issue":"3","key":"2021121022481954700_B9","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1159\/000101954","article-title":"Pulmonary involvement in Beh\u00e7et\u2019s disease.","volume":"75","author":"Uzun","year":"2008","journal-title":"Respiration"},{"issue":"1","key":"2021121022481954700_B10","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1097\/MD.0b013e318242ff37","article-title":"Pulmonary artery involvement and associated lung disease in Beh\u00e7et disease: a series of 47 patients.","volume":"91","author":"Seyahi","year":"2012","journal-title":"Medicine (Baltimore)"},{"issue":"3","key":"2021121022481954700_B11","first-page":"331","article-title":"Juvenile Beh\u00e7et\u2019s disease in Arab children.","volume":"14","author":"Bahabri","year":"1996","journal-title":"Clin Exp Rheumatol"},{"issue":"6","key":"2021121022481954700_B12","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1136\/annrheumdis-2015-208491","article-title":"Consensus classification criteria for paediatric Beh\u00e7et\u2019s disease from a prospective observational cohort: PEDBD.","volume":"75","author":"Kon\u00e9-Paut","year":"2016","journal-title":"Ann Rheum Dis"},{"issue":"1","key":"2021121022481954700_B13","first-page":"133","article-title":"Beh\u00e7et\u2019s disease in patients of German and Turkish origin living in Germany: a comparative analysis.","volume":"31","author":"K\u00f6tter","year":"2004","journal-title":"J Rheumatol"},{"issue":"4","key":"2021121022481954700_B14","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1016\/S0022-3476(98)70368-3","article-title":"Clinical features of Beh\u00e7et\u2019s disease in children: an international collaborative study of 86 cases.","volume":"132","author":"Kon\u00e9-Paut","year":"1998","journal-title":"J Pediatr"},{"issue":"1","key":"2021121022481954700_B15","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1097\/BOR.0b013e3283410088","article-title":"Major vessel involvement in Beh\u00e7et\u2019s disease: an update.","volume":"23","author":"Calamia","year":"2011","journal-title":"Curr Opin Rheumatol"},{"issue":"1","key":"2021121022481954700_B16","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1097\/BOR.0000000000000131","article-title":"Beh\u00e7et\u2019s syndrome: pulmonary vascular disease.","volume":"27","author":"Seyahi","year":"2015","journal-title":"Curr Opin Rheumatol"},{"issue":"11","key":"2021121022481954700_B17","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1016\/j.amjmed.2004.05.027","article-title":"Pulmonary artery aneurysms in Beh\u00e7et syndrome.","volume":"117","author":"Hamuryudan","year":"2004","journal-title":"Am J Med"},{"issue":"1","key":"2021121022481954700_B18","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1093\/rheumatology\/33.1.48","article-title":"Pulmonary arterial aneurysms in Beh\u00e7et\u2019s syndrome: a report of 24 cases.","volume":"33","author":"Hamuryudan","year":"1994","journal-title":"Br J Rheumatol"},{"issue":"3","key":"2021121022481954700_B19","doi-asserted-by":"crossref","first-page":"729","DOI":"10.2214\/ajr.172.3.10063870","article-title":"CT findings of pulmonary artery aneurysms during treatment for Beh\u00e7et\u2019s disease.","volume":"172","author":"Tunaci","year":"1999","journal-title":"AJR Am J Roentgenol"},{"issue":"1","key":"2021121022481954700_B20","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1097\/00005792-200301000-00006","article-title":"The long-term mortality and morbidity of Beh\u00e7et syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center.","volume":"82","author":"Kural-Seyahi","year":"2003","journal-title":"Medicine (Baltimore)"},{"issue":"2","key":"2021121022481954700_B21","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1378\/chest.118.2.479","article-title":"Intracardiac thrombus in Beh\u00e7et\u2019s disease: a systematic review.","volume":"118","author":"Mogulkoc","year":"2000","journal-title":"Chest"},{"issue":"6","key":"2021121022481954700_B22","doi-asserted-by":"crossref","first-page":"1199","DOI":"10.1160\/TH16-07-0529","article-title":"Pulmonary embolism and in situ pulmonary artery thrombosis in paediatrics. A systematic review.","volume":"117","author":"Rajpurkar","year":"2017","journal-title":"Thromb Haemost"},{"issue":"3","key":"2021121022481954700_B23","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1016\/S0022-3468(81)80700-2","article-title":"Pulmonary embolism in children.","volume":"16","author":"Buck","year":"1981","journal-title":"J Pediatr Surg"},{"issue":"5","key":"2021121022481954700_B24","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1016\/j.semarthrit.2014.10.014","article-title":"An outcome survey of 43 patients with Budd-Chiari syndrome due to Beh\u00e7et\u2019s syndrome followed up at a single, dedicated center.","volume":"44","author":"Seyahi","year":"2015","journal-title":"Semin Arthritis Rheum"},{"issue":"6","key":"2021121022481954700_B25","first-page":"808","article-title":"2018 update of the EULAR recommendations for the management of Beh\u00e7et\u2019s syndrome.","volume":"77","author":"Hatemi","year":"2018","journal-title":"Ann Rheum Dis"},{"issue":"2","key":"2021121022481954700_B26","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1024\/0301-1526.32.2.75","article-title":"Arterial lesions in Beh\u00e7et\u2019s disease.","volume":"32","author":"Saba","year":"2003","journal-title":"Vasa"},{"issue":"3","key":"2021121022481954700_B27","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1007\/s00417-014-2856-3","article-title":"Anti-interferon alpha antibodies and autoantibodies in patients with Beh\u00e7et\u2019s disease uveitis treated with recombinant human interferon alpha-2a.","volume":"253","author":"Aydinoglu-Candan","year":"2015","journal-title":"Graefes Arch Clin Exp Ophthalmol"},{"issue":"9","key":"2021121022481954700_B28","doi-asserted-by":"crossref","first-page":"2796","DOI":"10.1002\/art.27581","article-title":"Long-term remission after cessation of interferon-\u03b1 treatment in patients with severe uveitis due to Beh\u00e7et\u2019s disease.","volume":"62","author":"Deuter","year":"2010","journal-title":"Arthritis Rheum"},{"issue":"5","key":"2021121022481954700_B29","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1016\/j.semarthrit.2003.09.005","article-title":"Differential efficacy of human recombinant interferon-alpha2a on ocular and extraocular manifestations of Beh\u00e7et disease: results of an open 4-center trial.","volume":"33","author":"K\u00f6tter","year":"2004","journal-title":"Semin Arthritis Rheum"},{"issue":"4","key":"2021121022481954700_B30","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1001\/archderm.138.4.467","article-title":"Interferon alfa-2a in the treatment of Beh\u00e7et disease: a randomized placebo-controlled and double-blind study.","volume":"138","author":"Alpsoy","year":"2002","journal-title":"Arch Dermatol"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/doi\/10.1542\/peds.2019-0162\/1077373\/peds_20190162.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/doi\/10.1542\/peds.2019-0162\/1077373\/peds_20190162.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,11]],"date-time":"2021-12-11T07:47:55Z","timestamp":1639208875000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/144\/2\/e20190162\/38529\/The-Challenge-of-Treating-Pulmonary-Vasculitis-in"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,8,1]]},"references-count":30,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2019,8,1]]},"published-print":{"date-parts":[[2019,8,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2019-0162","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,8,1]]},"article-number":"e20190162"}}