Second European evidence-based consensus on the diagnosis and management of ulcerative colitis Part 3: Special situations

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Proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the procedure of choice for most patients with ulcerative colitis (UC) requiring colectomy.1 Pouchitis is a non-specific inflammation of the ileal reservoir and the most common complication of IPAA in patients with UC.2–7 Its frequency is related to the duration of follow up, occurring in up to 50% of patients 10 years after IPAA in large series from major referral centres.1–9 The cumulative incidence of pouchitis in patients with an IPAA for familial adenomatous polyposis is much lower, ranging from 0 to 10%.10–12 Reasons for the higher frequency of pouchitis in UC remain unknown. Whether pouchitis more commonly develops within the first years after IPAA or whether the risk continues to increase with longer follow up remains undefined. The diagnosis of pouchitis requires the presence of symptoms, together with characteristic endoscopic and histological abnormalities [EL3a, RG B]. Extensive UC, extraintestinal manifestations (i.e. PSC), being a non-smoker, p-ANCA positive serology and NSAID use are possible risk factors for pouchitis [EL3b, RG D] After proctocolectomy with IPAA, median stool frequency is 4 to 8 bowel movements,1–4,13,14 with about 700 mL of semiformed/liquid stool per day,2,13,14 compared to a volume of 200 mL/day in healthy people. Symptoms related to pouchitis include increased stool frequency and liquidity, abdominal cramping, urgency, tenesmus and pelvic discomfort.2,15 Rectal bleeding, fever, or extraintestinal manifestations may occur. Rectal bleeding is more often related to inflammation of the rectal cuff (“cuffitis,” Section 1.4),16 than to pouchitis. Faecal incontinence may occur in the absence of pouchitis after IPAA, but is more common in patients with pouchitis. Symptoms of pouch dysfunction in patients with IPAA may be caused by conditions other than pouchitis, including Crohn's disease of the pouch,17–19 cuffitis16 and an irritable pouch20 among other conditions. This is why the diagnosis depends on endoscopic and histological findings in conjunction with symptoms. Pouchoscopy and pouch mucosal biopsy should be performed in patients with symptoms compatible with pouchitis, in order to confirm the diagnosis.15,21 Patients with an ileoanal pouch occasionally have a stricture at the pouch-anal anastomosis, so a gastroscope rather than a colonoscope is preferred for pouchoscopy. Progression into the afferent ileal limb should always be attempted. Endoscopic findings compatible with pouchitis include diffuse erythema, which may be patchy, unlike that observed in UC. Characteristic endoscopic findings also include oedema, granularity, friability, spontaneous or contact bleeding, loss of vascular pattern, mucous exudates, haemorrhage, erosions and ulceration.17 Erosions and/or ulcers along the staple line do not necessarily indicate pouchitis.18,22,23 Biopsies should be taken from the pouch mucosa and from the afferent limb above the pouch, but not along the staple line. Histological findings of pouchitis are also non-specific, including acute inflammation with polymorphonuclear leukocyte infiltration, crypt abscesses and ulceration, in association with a chronic inflammatory infiltrate.22,23 There may be discrepancy between endoscopic and histologic findings in pouchitis, possibly related to sampling error.24,25 Morphological changes of the epithelium lining the ileal pouch normally develop in the 12–18 months after ileostomy closure, characterised by flattening and a reduced number, or complete disappearance of the villi, leading to villous atrophy (“colonic metaplasia”).23–25 Although the aetiology of pouchitis remains unknown, it can be inferred from the predeliction for patients with UC and the response to antibiotic therapy that the bacterial flora and/or other triggers of inflammation in UC are involved.26,27 Pouchitis tends to occur only after colonic metaplasia has developed in the pouch, although a causal association is unproven. The most frequent symptoms of pouchitis are increased number of liquid stools, urgency, abdominal cramping and pelvic discomfort. Fever and bleeding are rare [EL1c, RG B]. Routine pouchoscopy after clinical remission is not required [EL5, RG D] The clinical history and biopsies help discriminate between pouchitis, ischaemia, Crohn's disease (CD) and other rare forms of pouch dysfunction such as collagenous pouchitis, Clostridium difficile or cytomegalovirus pouchitis.28–30 Secondary pouchitis, caused by pelvic sepsis, usually causes focal inflammation and should be considered. Biopsies taken from the ileum above the pouch may reveal pre-pouch ileitis as a cause of pouch dysfunction, although this usually causes visible ulceration that may be confused with Crohn's disease.31 The possibility of non-specific ileitis caused by NSAIDs should be considered.32 The aetiology of pouchitis remains unclear. Risk factors, genetic associations, and serological markers of pouchitis suggest that a close interaction between the host immune response and the pouch microbiota plays a relevant role in the aetiology of this idiopathic inflammatory condition.33 Reported risk factors for pouchitis include extraintestinal manifestations being a and use of and the presence of are also with pouchitis. are with to the role of risk of the on risk factors from the and patients as healthy pouchitis Crohn's disease or irritable pouch The risk of pouchitis increased the for IPAA the NSAIDs or The risk of a diagnosis of Crohn's disease in the pouch increased by being a and increased with a pouch of duration with symptoms of and a pouch is although is a common cause of pouch dysfunction other causes a volume pouch, and pouch have and are The risk is the use of or which that may have irritable bowel to symptoms of colitis pouch to irritable bowel a has in The has that factors may pouchitis. disease to the extraintestinal and with risk factors should not proctocolectomy is but should be with the and is a between and endoscopic or the is on the risk of pouch dysfunction after IPAA a has it is to the higher risk of pouchitis. are of of and factors for pouchitis or irritable pouch should not be as for pouch the of symptoms and pouchitis can be into remission pouch or pouchitis frequency with endoscopic and with pouchitis may be into acute or on the The for is a duration of 4 to of patients develop chronic pouchitis requiring and a has pouchitis to pouchitis may also be idiopathic in remission per per Pouchitis may also be on the response to antibiotic for antibiotic to The Pouchitis has developed to and the of The is a that symptoms, and of the has a of Patients with a are as pouchitis although a should clinical symptoms and endoscopic or histological of pouchitis. 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