In Germany, about 150,000 people are affected by ulcerative colitis. For most patients, the disease begins during schooling or vocational training and continues throughout life. Ulcerative colitis is a chronic inflammatory disease of the colon mucosa, which manifests itself primarily in the distal rectum. After its onset in the rectum (proctitis), it spreads continuously proximally.
The clinical classification (Silverberg MS et al. 2005) is based on the spread type:
Several score systems are available for the macroscopic-endoscopic classification of ulcerative colitis, such as the partial Mayo-Score:
The incidence of ulcerative colitis in Germany is about 4/100 000 inhabitants/year. The prevalence is assumed to be about 90/100 000 inhabitants/year. Incidence and prevalence do not seem to have changed in recent decades, although an increase is observed worldwide. Within Europe, however, there is a north-south divide, with the highest incidence in Iceland (24.3/100 000) and the lowest in Portugal (1.7/100 000). The risk of disease for siblings of patients with CU is 15 times higher than for the general population (Vermeire S 2006). However, the lifetime risk of developing ulcerative colitis for first-degree relatives is only about 5%. This is important information for patients in family planning. Familial cases of ulcerative colitis seem to affect the female sex more, and the age of onset of the disease seems to be lower.
The exact triggers and mechanisms are only partially known. Apparently, several extrinsic and intrinsic factors interact in the triggering process.
m:w=1:1; in terms of age-specific incidence, the highest rate is in the group of 25-35 year-olds (approx. 4.5/100 000). A second frequency peak is found in the age group > 55 years.
The natural course of ulcerative colitis is characterized by episodes of disease relapses alternating with phases of remission. The main symptoms are bloody-mucus diarrhoea, possibly fever, anaemia; furthermore, sometimes cramp-like pain in the left lower abdomen (usually before defecation). A simple but still valid clinical definition of severe colitis was established by Truelove and Witts as early as 1954:
The remission is characterized by the combination
Extraintestinal symptoms (about 30% of patients):
Skin changes and changes in the mucous membranes close to the skin in ulcerative colitis (10%):
Endoscopically, ulcerative colitis presents as a continuous inflammation of varying severity spreading from the rectum to the oral cavity. The spectrum ranges from low activity with brittle, granular mucosa with reduced vascular drawing and possibly mild erythema to severe activity in the form of (confluent) ulcers and spontaneous, predominantly petechial haemorrhages. The transition from inconspicuous to inflamed mucosa is typically a sharp line. After anal, the inflammation typically increases in severity. In the case of prolonged severe activity, pseudopolyps may occur.
The endoscopic activity correlates well with the severity of the inflammation. To assess activity, endoscopic visualization of the right transverse colon is usually sufficient. Especially in severe colitis, further prophylaxis should be avoided because of the risk of perforation and worsening of the flare. Long-term disease activity may result in structural damage of varying degrees.
The spectrum ranges from individual scarred areas of mucous membrane to the complete loss of homologation with a rigid colon resembling a bicycle inner tube
Note: In everyday clinical practice, the question regularly arises at what points in the course of the patient's life the endoscopy should be performed.
Transabdominal ultrasound: method with the advantage of non-invasivity. Inflammations in the area of the colon can be detected with a sensitivity of up to 90%. In the hands of a trained examiner, both the activity of the disease and the extent of the disease can be reliably determined. In severe ulcerative colitis there is moderate wall thickening (usually 4-6 mm) with preserved wall stratification and hyperperfusion limited to the mucosa. An echoric environmental reaction should rather remind of Crohn's disease, but is possible in severe colitis and especially in toxic megacolon and must then be understood as an alarm signal.
CT and MRI: Supplementary sectional imaging procedures such as computed tomography and magnetic resonance imaging are generally used for differential diagnosis of Crohn's disease. If ulcerative colitis has been chronically active for many years, scarring caused structural damage to the colonic wall can be seen as a loss of home-trition (so-called "bicycle inner tube phenomenon"). This can be detected sonographically as well as by computer and MR-tomography.
X-ray with double contrast enema: loss of homophoria ("bicycle inner tube" phenomenon) "collar button fulcera".
Serological laboratory values may be normal, especially in mild to moderate ulcerative colitis and/or distal colitis. In proctitis, the C-reactive protein correlates with the extent of the disease and clinical activity. In patients with severe disease activity, SPA elevations and anemia are found.
Faecal stool markers, especially calprotectin, lactoferrin, lysozyme, PMN-elastase, can be used as markers of clinical inflammatory activity in ulcerative colitis. Calprotectin has the highest sensitivity (Herrlinger et al. 2004)
Stool cultures are necessary for the differentiation and diagnosis of self-limiting infectious colitis stool cultures.
Histopathology: The initial diagnosis should be made on at least 2 step biopsies from the ileum and from all colon sections including the rectum. Histopathology: Diffuse panmucosal chronic inflammation (lymphocytes and plasma cells) in combination with a disturbance of the crypt architecture/crypt atrophy, plasmocytosis in the basal mucosal stroma Paneth-cell metaplasia distal to the right colonic flexure, reduction of the number of goblet cells or the mucin content of the single cells, continuous distribution of the inflammatory and structural mucosal changes, decreasing gradient from distal to proximal.
The histopathological evaluation of inflammatory activities in ulcerative colitis is based on the extent of tissue infiltration by segment-nucleated neutrophil granulocytes as well as damage to the intestinal epithelium, the extent of cryptitic lesions and cryptitic abscesses and the extent of erosive and ulcerative lesions. The utility of histopathology for predicting recurrence and assessing adequate control of inflammation has implications for therapeutic management and reduction of neoplasia risk (Winther KV et al 2004; Lutgens MW et al 2008).
The diagnosis of ulcerative colitis is based on the detection of a characteristic combination of clinical, laboratory, endoscopic, radiological and pathomorphological findings. There is no gold standard for diagnosis. Pathohistological diagnosis is also based on the synoptic assessment of a combination of characteristics, mainly concerning the type and distribution of inflammatory infiltrates and changes in the mucosal architecture. The individual histological findings are not specific in themselves and may also occur in other forms of inflammatory bowel disease (Kucharzik T et al. 2019).
Normal findings in the histopathology of mucosal biopsies exclude active ulcerative colitis! The initial diagnosis should be made on at least 2 step biopsies from the ileum and from all colon sections including the rectum.
Note: If an exact assignment to the entities ulcerative colitis or Crohn's disease is not possible, the clinical picture is listed under the diagnosis "indeterminate colitis" (Inflammatory Bowel Disease unclassified (Silverberg).
Growth disorders in childhood
Weight loss
Recurrent, partly massive bleeding
Toxic colon dilatation (toxic megacolon) with high lethality (30%)
Colorectal carcinoma: Occurs mostly after the 10th year of illness. A significant proportion of colorectal carcinomas can also occur before the 8th year of illness (Winther KV et al. 2004). In one study it could be shown that 22 % of patients with ulcerative colitis had already developed the disease before the start of regular monitoring colonoscopies (Lutgens MW et al. 2008). The cumulative colorectal carcinoma risk within a 10-year period is 1 %, within a 20-year period 3 %, within a 30-year period 7 % (Annese V et al. 2013).
The development of carcinoma in chronic ulcerative colitis often occurs without a preliminary polyp stage at the base of an intraepithelial neoplasia (IEN)
Rare late complication: Amyloidosis
A causal, drug therapy for ulcerative colitis is not yet available. Proctocolectomy is a curative therapy option, but it is associated with considerable defect formation and often functional limitations. All drug therapies are aimed at modifying the immunological response. The therapeutic decision depends on the activity of the disease, the localisation, the course, the previous therapeutic modalities and possible complications or extraintestinal manifestations.
Ulcerative proctitis: The standard therapy for proctitis consists of topical therapy with 5-ASA suppositories (1 g/d). Mesalazine foams or enemas are considered equivalent (recommended daily dose ≥ 2 g/d; therapy is best administered at night). However, compared to suppositories, they are often less well tolerated by patients. In case of lack of improvement, topical 5-ASA therapy should be combined with oral 5-ASA therapy (dose ≥ 3 g/d) or a topical corticosteroid (e.g. budesonide 2 mg/d, hydrocortisone acetate 100 mg/d, or beclometasone 3 mg/d). Oral 5-ASA monotherapy should not be used primarily because it is inferior to topical therapy. If patients do not tolerate topical therapy, oral 5-ASA therapy can be tried with ≥ 3 g/d.
Left-sided colitis: If the course is uncomplicated, initial therapy with topical mesalazine foams or enemas in a dose of 2-4 g/d - (therapy is best performed at night). Combined 5-ASA therapy is increasingly used as standard therapy for left-sided colitis: oral administration (recommended minimum dose > 3 g/d) and enemas (recommended dose at least 1 g/d). Response to therapy with discontinuation of hematochezia should be within 14 days. If this effect does not occur, systemic steroid therapy should be initiated. Different regimens are used, the minimum starting dose should be 40 mg prednisolone equivalent (typically 1 mg/kg body weight) at ≥. The duration of therapy should not be less than 4 weeks with a sneak-out regimen due to an increased relapse rate. Note: Daily doses ≤ 15 mg prednisolone equivalent are considered ineffective in active ulcerative colitis. Note: It is very important to evaluate the clinical response on days 7-10, as a steroid-refractory course is present if taken correctly and there is no response, which requires a rapid change in therapy.
Extensive colitis: Extensive colitis with mild to moderate disease activity should be treated by combined 5-ASA administration in the same way as left-sided colitis. The same recommendations regarding dose and frequency of application apply.
Severe ulcerative colitis Patients with severe colitis as defined by Truleove and Witts are usually at acute risk and usually require hospital admission and immediate therapy: The general consensus is an intravenous steroid therapy with 1 mg/kg bw prednisolone as an i.v. bolus 1 ×/d. Higher steroid doses are not more effective, lower doses are less effective. The success of the therapy should be assessed on day 3 and the duration of therapy should be limited to 7-10 d. It has been shown that longer therapy does not promise any advantage in the event of a lack of response (25-34% of patients).
Ulcerative colitis with steroid-dependent course: Corticosteroids must not be used to maintain remission due to considerable long-term side effects. Thiopurines, TNF-α antibodies, vedolizumab and tofacitinib are effective alternatives. TNF-α antibodies or vedolizumab are used for relapse activity under azathioprine therapy (2-2.5 mg/kg daily dose over at least 12 weeks); alternatively:tofacitinib.
Maintenance of remission in ulcerative colitis: most patients experience a recurrence within 12 months. In this respect, remission-maintaining therapy is recommended for all patients (current ECCO guideline) with the aim of achieving steroid-free remission.
Surgical therapy: The standard surgical procedure for refractory or complicative ulcerative colitis is laparoscopic proctocolectomy with ileoanal pouch. The operation should be performed as a two- or three-stage operation. Approximately 15-35 % of patients with ulcerative colitis will have to undergo proctocolectomy during their lifetime. However, especially young patients will be advised to undergo surgery because of the longer life expectancy and the associated higher probability of later severe dysplasia.
There are three main indications for proctocolectomy:
Stenoses in confirmed ulcerative colitis are often caused by a stenosing carcinoma, so that proctocolectomy should also be performed. If the proctocolectomy is performed due to dysplasia or malignoma, an annual endoscopic check of the pouch is recommended.
Surgery as an emergency indication: Emergency proctocolectomies are still associated with high morbidity (27-51%) and mortality (5-8%). Therapy-refractory bleeding is an emergency indication for surgery: vital threatening colon bleeding with the necessity of transfusion of > 4 red blood cell concentrates in 24 h. The most severe form of acute, severe colitis is toxic megacolon. It can take a life-threatening course. This situation is defined by a septic clinical picture with dilatation of the transverse colon to > 5.5 cm. In the context of severe inflammatory activity, free colon perforation may occur. This is treated by emergency colectomy, leaving the rectum intact.
The average life expectancy of all patients with ulcerative colitis corresponds to the life expectancy of the normal population. However, due to disease-related complaints, there may be:
Transdermally applied nicotine: In randomized placebo-controlled clinical trials in patients with active ulcerative colitis, transdermal nicotine was shown to produce additional beneficial effects in the treatment of active ulcerative colitis in combination with standard therapy (Sandborn WJ et al. 1997). However, the administration of transdermal nicotine leads to frequent and sometimes severe side effects, especially in lifelong nonsmokers.
see also under microbiological therapy
Langhorst J et al. Results of a 12-month, randomized, prospective, double-blind, double-dummy, active-controlled study of the efficacy and safety of the phytotherapeutic MYRRHINIL-INTEST® compared with mesalazine in clinical remission maintenance of patients with ulcerative colitisZ Gastroenterol; 49 - P090 . DOI: 10.1055/s-0031-1285362