This is the first European Crohn’s and Colitis Organisation [ECCO] consensus guideline that addresses extra-intestinal manifestations [EIMs] in inflammatory bowel disease [IBD]. It has been drafted by 21 ECCO members from 13 European countries. Although this is the first ECCO consensus guideline that primarily addresses EIMs, it is partly derived from, updates, and replaces previous ECCO consensus advice on EIMs, contained within the consensus guidelines for Crohn’s disease [CD] and ulcerative colitis [UC]. The strategy to define consensus was similar to that previously described in other ECCO consensus guidelines [available at www.ecco-ibd.eu]. Briefly, topics were selected by the ECCO guidelines committee [GuiCom]. ECCO members were selected to form working groups. Provisional ECCO Statements and supporting text were written following a comprehensive literature review, then refined following two voting rounds which included national representative participation by ECCO’s 35 member countries. The level of evidence was graded according to the Oxford Centre for Evidence-based Medicine [www.cebm.net]. The ECCO Statements were finalised by the authors at a meeting in Vienna in October 2014 and represent consensus with agreement of at least 80% of participants. Complete consensus [100% agreement] was reached for most statements. The supporting text was then finalised under the direction of each working group leader [VA, SV, FC, MH] before being integrated by the two consensus leaders [MH, FC]. This consensus guideline is pictorially represented within the freely available ECCO e-Guide [http://www.e-guide.ecco-ibd.eu/].
Up to 50% of patients with inflammatory bowel disease [IBD] experience at least one extra-intestinal manifestation [EIM], which can present before IBD is diagnosed. EIMs adversely impact upon patients’ quality of life and some, such as primary sclerosing cholangitis [PSC] or venous thromboembolism [VTE], can be life-threatening. The probability of developing EIMs increases with disease duration and in patients who already have one EIM. EIMs are more common in CD than UC, particularly in patients with colonic CD; some EIMs, such as iritis/uveitis, are more common in women whereas PSC and ankylosing spondylitis are more common in males. Most EIMs run in parallel with intestinal disease activity, with the exception of ankylosing spondylitis and uveitis and with uncertainty regarding PSC and pyoderma gangrenosum [PG]. The management of complex EIMs should be discussed in a multidisciplinary team meeting.
Keywords: acute febrile neutrophilic dermatosis; acute pancreatitis in inflammatory bowel disease; anti TNF; arthritis; arthropathy; aural desease; bronchiectasis; broncho-pulmonary manifestations specific to inflammatory bowel disease; cardiovascular manifestations of inflammatory bowel disease; chronic bronchitis; chronic pancreatitis; ciclosporin A; coagulopathy in inflammatory bowel disease; consensus paper; dermatological drug adverse events; desquamative interstitial pneumonia; diffuse pan-bronchiolitis; drug-induced liver injury; drug-induced manifestations; eosinophilic interstitial pneumonia; erythema nodosum; extra-intestinal manifestations; eye disease; granulomatous bronchiolitis; granulomatous hepatitis; granulomatous interstitial lung disease; guideline; hepatic abscess; hepatic amyloidosis; hepato-pancreato-biliary disease; inflammatory bowel disease; investigation and diagnostic criteria; lymphocytic interstitial pneumonia; metabolic bone disease; methotrexate; metronidazole; nasal disease; natalizumab; natural history; neurological disease; non-alcoholic fatty liver disease; non-PSC liver disease in inflammatory bowel disease; oral disease; organising pneumonia; pathogenesis; portal vein thrombosis; Primary Sclerosing cholangitis; pulmonary manifestations of inflammatory bowel disease; pyoderma gangrenosum; skin disease; sulfasalazine; Sweet syndrome; thalidomide; thiopurines; urogenital manifestations of inflammatory bowel disease; usual interstitial pneumonia; uveitis; vedolizumab