{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T03:42:24Z","timestamp":1775706144486,"version":"3.50.1"},"reference-count":16,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,2,28]],"date-time":"2014-02-28T00:00:00Z","timestamp":1393545600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2014,3]]},"abstract":"Abstract<\/jats:title>Adult\u2010onset Still's Disease (AOSD<\/jats:styled-content>), often though as the adult variant of systemic juvenile idiopathic arthritis (JIA<\/jats:styled-content>), has an incidence of 1\u20133 cases per 1 million. Cardinal manifestations include fever, arthritis, skin rash, sore throat, hepatosplenomegaly and lymphadenopathy. Prolongation in diagnosing this disease results from its similarity to infectious, malignant and rheumatic diseases and lack of biomarkers. Pulmonary arterial hypertension (PAH<\/jats:styled-content>) is a rare pulmonary complication of AOSD<\/jats:styled-content>, and we are aware of only six cases reported in literature to date. Here we present a patient with AOSD<\/jats:styled-content> who has developed pulmonary hypertension as a complication. We report a case of AOSD<\/jats:styled-content> complicated by PAH<\/jats:styled-content> treated successfully with tocilizumab, a humanized monoclonal antibody to human interleukin (IL<\/jats:styled-content>)\u20106 receptor. A Pubmed and Medline search for evidence of pulmonary hypertension in AOSD<\/jats:styled-content> and use of IL<\/jats:styled-content>\u20106 inhibition in management was performed. Data for this study was collected from the patient's chart records. No infectious or neoplastic cause of her disease was identified and after extensive diagnostic workup, the patient was diagnosed with AOSD<\/jats:styled-content> fulfilling Yamaguchi criteria. After initiation of IL\u20106 therapy the patient was followed over time to monitor the hemodynamic changes in pulmonary vasculature. Following treatment with Tocilizumab, the patient showed dramatic improvement in her clinical symptoms and remains in remission, through combination of tocilizumab (8\u00a0mg\/kg), methotrexate and prednisone. Improvement of systemic symptoms, right heart catheterization (RHC<\/jats:styled-content>) findings and the VECTRA\u2010DA<\/jats:styled-content> score served as a measure of treatment response. Tocilizumab has been effective in demonstrating marked improvement in both the clinical and laboratory parameters. Tocilizumab is an effective novel treatment for AOSD<\/jats:styled-content> with PAH<\/jats:styled-content>. This is the first documented report of successful use of tocilizumab in AOSD<\/jats:styled-content> patients presenting with PAH<\/jats:styled-content>. Prospective comparative studies could help validate its efficacy and safety.<\/jats:p>","DOI":"10.1111\/1756-185x.12324","type":"journal-article","created":{"date-parts":[[2014,3,3]],"date-time":"2014-03-03T08:59:13Z","timestamp":1393837153000},"page":"336-340","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":39,"title":["A novel therapeutic approach in pulmonary arterial hypertension as a complication of adult\u2010onset Still's disease: targeting IL<\/scp>\u20106"],"prefix":"10.1111","volume":"17","author":[{"given":"Sabeeda","family":"Kadavath","sequence":"first","affiliation":[{"name":"Rheumatology Division Lincoln Medical and Mental Health Center New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ekaterini","family":"Zapantis","sequence":"additional","affiliation":[{"name":"North Shore\u2010LIJ Health System New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald","family":"Zolty","sequence":"additional","affiliation":[{"name":"Department of Medicine (Cardiology) Albert Einstein College of Medicine of Yeshiwa University New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petros","family":"Efthimiou","sequence":"additional","affiliation":[{"name":"Rheumatology Division Lincoln Medical and Mental Health Center New York New York USA"},{"name":"Weill Cornell Medical College New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,2,28]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2007.04.002"},{"key":"e_1_2_6_3_1","first-page":"2189","article-title":"Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still's disease","volume":"31","author":"Chen DY","year":"2004","journal-title":"J Rheumatol"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keq284"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2003.013680"},{"key":"e_1_2_6_6_1","first-page":"2422","article-title":"Serum cytokine profiles in patients with adult onset Still's disease","volume":"30","author":"Choi JH","year":"2003","journal-title":"J Rheumatol"},{"key":"e_1_2_6_7_1","first-page":"653","article-title":"Interleukin 6: a possible marker of disease activity in adult onset Still's disease","volume":"14","author":"Scheinberg MA","year":"1996","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1155\/2012\/156890"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmr.2009.11.013"},{"key":"e_1_2_6_10_1","first-page":"275","article-title":"Exuberant endothelial cell growth and elements of inflammation are present in plexiform lesions of pulmonary hypertension","volume":"144","author":"Tuder RM","year":"1994","journal-title":"Am J Pathol"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)65137-1"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V74.1.1.1"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0065-2776(08)60532-5"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.immunol.23.021704.115806"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2008.092932"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(08)60454-7"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-12-4602"}],"container-title":["International Journal of Rheumatic Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2F1756-185X.12324","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/1756-185X.12324","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T18:04:40Z","timestamp":1695492280000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/1756-185X.12324"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,2,28]]},"references-count":16,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2014,3]]}},"alternative-id":["10.1111\/1756-185X.12324"],"URL":"https:\/\/doi.org\/10.1111\/1756-185x.12324","archive":["Portico"],"relation":{},"ISSN":["1756-1841","1756-185X"],"issn-type":[{"value":"1756-1841","type":"print"},{"value":"1756-185X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,2,28]]},"assertion":[{"value":"2014-02-28","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}