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We systematically reviewed the literature evidence to evaluate tools and investigations used for the diagnosis and for the assessment of the treatment response in inflammatory bowel disease patients with extraintestinal manifestations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We searched in PubMed, Embase and Web of Science from January 1999\u2013December 2019 for all interventional and non\u2010interventional studies published in English assessing diagnostic tools and investigations used in inflammatory bowel disease patients with extraintestinal manifestations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010five studies (16\u00a0interventional and 29\u00a0non\u2010interventional) were included in our systematic review, enrolling 7994\u00a0inflammatory bowel disease patients. The diagnostic assessment of extraintestinal manifestations was performed by dedicated specialists in a percentage of cases ranging from 60\u2013100% depending on the specific condition. The clinical examination was the most frequent diagnostic strategy, accounting for 35 studies (77.8%). In patients with primary sclerosing cholangitis or rheumatological symptoms, biochemical and imaging tests were also performed. Anti\u2010TNF agents were the most used biological drugs for the treatment of extraintestinal manifestations (20 studies, 44.4%), and the treatment response varied from 59.1% in axial spondyloarthritis to 88.9% in ocular manifestations. No benefit was detected in primary sclerosing cholangitis patients after treatment with biologics.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In the clinical management of inflammatory bowel disease patients with extraintestinal manifestations the collaboration of dedicated specialists for diagnostic investigations and follow\u2010up is key to ensure the best of care approach. However, international guidelines are needed to homogenise and standardise the assessment of extraintestinal manifestations.<\/jats:p><\/jats:sec>","DOI":"10.1177\/2050640620950093","type":"journal-article","created":{"date-parts":[[2020,8,11]],"date-time":"2020-08-11T05:20:34Z","timestamp":1597123234000},"page":"1013-1030","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":31,"title":["Assessment of extraintestinal manifestations in inflammatory bowel diseases: A systematic review and a proposed guide for clinical trials"],"prefix":"10.1002","volume":"8","author":[{"given":"Lucas","family":"Guillo","sequence":"first","affiliation":[{"name":"Department of Gastroenterology University Hospital of Marseille Nord University of Aix\u2010Marseille Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ferdinando","family":"D\u2019Amico","sequence":"additional","affiliation":[{"name":"Department of 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