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01092014-GUT.Pdf Downloaded from gut.bmj.com on August 6, 2014 - Published by group.bmj.com Guidelines A global consensus on the classification, diagnosis and multidisciplinary treatment of perianal fistulising Crohn’s disease Editor’s choice Scan to access more free content Krisztina B Gecse,1,2 Willem Bemelman,3 Michael A Kamm,4 Jaap Stoker,5 Reena Khanna,6,7 Siew C Ng,8 Julián Panés,9 Gert van Assche,10 Zhanju Liu,11 Ailsa Hart,12,13 Barrett G Levesque,14,15 Geert D’Haens,1,2 for the World Gastroenterology Organization, International Organisation for Inflammatory Bowel Diseases IOIBD, European Society of Coloproctology and Robarts Clinical Trials ▸ Additional material is ABSTRACT and treatment of pCD, based on best available evi- published online only. To view Objective To develop a consensus on the dence and expert opinion in order to offer guid- please visit the journal online fi (http://dx.doi.org/10.1136/ classi cation, diagnosis and multidisciplinary treatment ance to clinicians. gutjnl-2013-306709). of perianal fistulising Crohn’s disease (pCD), based on best available evidence. For numbered affiliations see METHODS end of article. Methods Based on a systematic literature review, Working group statements were formed, discussed and approved in The World Congress of Gastroenterology 2013 Correspondence to multiple rounds by the 20 working group participants. called for the development of state-of-the-art, evi- Professor Geert D’Haens, fi Consensus was de ned as at least 80% agreement dence based views for designated areas of gastro- Department of fi Gastroenterology and among voters. Evidence was assessed using the modi ed enterology. An expert consensus group on pCD Hepatology, Academic Medical GRADE (Grading of Recommendations Assessment, was formed and a literature review process (sum- Center, University of Development, and Evaluation) criteria. marised in online supplementary figure S1) fol- Amsterdam, Meibergdreef 9, Results Highest diagnostic accuracy can only be Amsterdam 1105 AZ, lowed, covering all relevant papers and abstracts established if a combination of modalities is used. until March 2014. The search initially identified The Netherlands; fi [email protected] Drainage of sepsis is always rst line therapy before 4680 references. A selection process by the research initiating immunosuppressive treatment. Mucosal healing committee members and additional references iden- Received 3 January 2014 is the goal in the presence of proctitis. Whereas tified by manual search led to 247 retained articles. Revised 17 May 2014 antibiotics and thiopurines have a role as adjunctive Accepted 28 May 2014 Published Online First treatments in pCD, anti-tumour necrosis factor (anti-TNF) is the current gold standard. The efficacy of infliximab is Consensus process 20 June 2014 fi fi best documented although adalimumab and Four areas of interest were identi ed: (1) classi ca- certolizumab pegol are moderately effective. Oral tion and scoring, (2) diagnosis, (3) medical treat- tacrolimus could be used in patients failing anti-TNF ment, and (4) surgical management. For each of therapy. Definite surgical repair is only of consideration these areas the research committee drafted state- in the absence of luminal inflammation. ments based on the systematic literature review and Conclusions Based on a multidisciplinary approach, developed potential algorithms. These were dis- items relevant for fistula management were identified cussed and revised by the group during their meet- fi and algorithms on diagnosis and treatment of pCD were ings. As a nal step all participants voted on each fi developed. statement, using de nitions of agreement previ- ously reported by Bitton et al.4 Consensus was a priori defined as at least 80% agreement. The fi INTRODUCTION modi ed GRADE (Grading of Recommendations Penner and Crohn first described perianal fistulas as Assessment, Development, and Evaluation) criteria complications of Crohn’s disease (CD) 75 years were used to establish the strength of recommenda- ago.1 Population based studies confirmed that peri- tions and the quality of evidence (see online sup- 5 anal fistulas are the most common manifestation of plementary table S1). fistulising CD, developing in 20% of Crohn’s patients and recurring in approximately 30% of the CONSENSUS STATEMENTS cases.23The cumulative incidence of perianal fistu- Section 1: Classification and scoring lising CD (pCD) is 12% after 1 year and this Statement 1—General considerations for doubles 20 years after diagnosis.2 The risk of devel- classification and scoring oping a fistula depends on disease location, being 1.1 A clinically useful classification of perianal most frequent in colonic disease with rectal fistulas in CD should enable the treating physician involvement.3 Perianal fistulas impose a significant to determine optimal management strategy. burden on the patients. Unfortunately there are Vote: A+=80%, A=15%, D=5%; grade of recom- To cite: Gecse KB, limitations in the available literature, making it mendation: 1C Bemelman W, Kamm MA, challenging to develop an evidence based approach 1.2 Scoring of perianal fistula activity in CD should et al. Gut 2014;63: to pCD. The objective of the working group was to enable the treating physician to evaluate disease – 1381 1392. develop a consensus on the classification, diagnosis severity and response to therapy. Gecse KB, et al. Gut 2014;63:1381–1392. doi:10.1136/gutjnl-2013-306709 1381 Downloaded from gut.bmj.com on August 6, 2014 - Published by group.bmj.com Guidelines Vote: A+=70%, A=20%, A−=5%, D−=5%; grade of recom- (r=0.371, p=0.036).22 One year of infliximab therapy was asso- mendation: 1C ciated with significant improvement of the Van Assche score, particularly for the item T2 hyperintensity.23 Disappearance of Discussion Several classification and scoring systems have been contrast enhancement was the only MR feature associated with developed in an attempt to quantify disease extent and severity 23 – clinical remission. In a more recent study, this score was found of pCD.6 16 We propose that a distinction is made between a to be insensitive to change in patients with reduced fistula detailed anatomic description of perianal fistulas (ie, classifica- calibre during long term follow-up.21 Recently, perianal fistula tion) and the assessment of fistula activity (scoring), a dynamic length assessed by MRI was found to be a predictor of response measure that is sensitive to change under treatment. However, to treatment.24 In conclusion, improved instruments to score both components are necessary to design an optimal therapeutic perianal CD fistula activity are needed. strategy and are relevant for prognosis (table 1). Statement 3—Fistula anatomy Statement 2—Fistula activity 3.1 The course of the fistula tract in relation to the anal sphinc- Evaluation of fistula activity is recommended both by clinical ter and the levator plate (superficial, intersphincteric, trans- and radiological (MRI) features. sphincteric, suprasphincteric, extrasphincteric, and supralevator Vote: A+=70%, A=20%, A−=5%, D+=5%; grade of recom- or infralevator) is an important component for classification. mendation: 2C Vote: A+=90%, A=5%, D−=5%; grade of recommendation: 2B Discussion The Perianal Disease Activity Index (PDAI) is based 3.2 High and low transsphincteric fistulas should be distinguished on the assessment of quality of life (pain/restriction of activities in the classification. Fistulas are defined as low when the tract runs and restriction of sexual activities) and perianal disease severity through the lower one third of the external anal sphincter. (fistula discharge, type of perianal disease, and degree of indur- Vote: A+=70%, A=20%, A−=10%; grade of recommendation: 2C ation) rated on a five-point Likert scale.13 It was tested for reli- Discussion Parks et al6 studied the anatomy of perianal fistulas ability and responsiveness and was validated against physicians’ and the associated risk of post-surgical incontinence in 400 con- and patients’ global assessments.13 A cut-off PDAI score >4 secutive patients, as illustrated in figure 1. Section 4 describes resulted in 87% accuracy when using clinical assessment (active the impact of fistula anatomy on surgical treatment. fistula drainage and/or signs of local inflammation) as refer- ence.17 ‘Fistula drainage assessment’ was first used to quantify — fistula healing in a randomised controlled trial (RCT) with Statement 4 Proctitis fi infliximab.14 A fistula was considered ‘closed’ when it no longer The presence of proctitis, de ned as any ulceration and/or stric- fl drained despite gentle finger compression. A response was ture in the rectum, or in ammation and/or stricture of the anal fi defined as a reduction of 50% or more in the number of drain- canal, is an important component for stula assessment. ing fistulas on at least two consecutives visits. Fistula remission Vote: A+=90%, A=10%; grade of recommendation: 1C fi fi was de ned as the absence of any draining stulas on two con- Discussion The presence of proctitis is highly relevant for secutive visits. Although this clinical assessment has been used fistula management and prognosis as discussed in detail in sections 18–20 ‘ fi in further RCTs, it has several drawbacks. Gentle nger 3and4. compression’ is largely investigator dependent and is an approach that has never been formally validated. Persistent Statement 5—Abscess tracts without fluid drainage are scored as ‘remission’. A perianal abscess clinically defined as fluctuation and radio- Moreover, the external appearance of the fistula is used as a sur- logically defined as a confined fluid collection (a hyperintense rogate for the whole internal fistula tract. MRI studies have lesion on T2 weighted MRI images and/or a hypo- or anechoic shown that internal fistula healing lags behind clinical remission area with endoanal ultrasound (EUS)) with a rim of inflamma- by a median of 12 months.21 The site of a former/residual tory tissue (rim enhancement on post-contrast T1 weighted fistula tract could be the path of least resistance for a new or MRI images and/or often poorly demarcated lesions on EUS) is recurrent fistula tract to occur.22 In an attempt to combine ana- an important component for classification.
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