*Sławomir Glinkowski, Daria Marcinkowska
Ulcerative colitis: assessment of disease activity based on contemporary scales
Wrzodziejące zapalenie jelita grubego – ocena aktywności choroby na podstawie współcześnie stosowanych skal
Department of General and Oncological Surgery, Health Centre in Tomaszów Mazowiecki
Head of Department: Włodzimierz Koptas, MD, PhD
Streszczenie
Wrzodziejące zapalenie jelita grubego jest chorobą budzącą coraz większe zainteresowanie wśród społeczeństwa. Tryb życia oraz dieta sprawiają, iż coraz więcej pacjentów skarży się na dolegliwości ze strony przewodu pokarmowego. Zachorowalność na WZJG od kilku lat nie ulega wzrostowi, jednak wciąż pojawiają się nowe substancje lecznicze oraz możliwości leczenia. Aby wystandaryzować ocenę pacjentów z WZJG powstało i nadal powstaje wiele skal oceniających zarówno stan kliniczny, jak i zmiany w obrazie endoskopowym czy stopień odżywienia. Pierwsza skala oceniająca ciężkość rzutów WZJG, do dziś stosowana, powstała już w 1955 roku i została stworzona przez Truelove’a i Wittsa podczas badania wpływu kortyzonu na przebieg WZJG. Aktualnie, wciąż powstają nowe skale, które mają za zadanie w jak największym stopniu ułatwić i ujednolicić podejmowanie decyzji terapeutycznych przez lekarzy prowadzących chorych na WZJG. Ze względu na rozpowszechnienie choroby, chorzy ci nie zawsze trafiają jedynie do ośrodków referencyjnych, ale również do mniejszych szpitali niespecjalizujących się w terapii pacjentów cierpiących na WZJG. Z punktu widzenia lekarza chirurga najważniejszym jest ocena, czy pacjent wymaga operacji, czy możliwe jest postępowanie oparte jedynie na farmakoterapii. Gwałtowny przebieg choroby, znaczne nasilenie dolegliwości oraz niedożywienie nierzadko prowadzące do wyniszczenia uniemożliwiają leczenie zachowawcze, zmuszając do podjęcia decyzji o bardziej radykalnym postępowaniu i interwencji chirurgicznej.
Summary
Ulcerative colitis is a disease which has been attracting a growing interest in society. The contemporary lifestyle and diet cause an increasing number of patients to complain of gastrointestinal problems. The incidence of ulcerative colitis has not increased over the last few years; however, new medicinal substances and treatment possibilities have still been occurring. In order to standardise the assessment of patients with ulcerative colitis a number of scales have been developed for the assessment of their clinical status as well as changes in the endoscopic picture and nutritional status. The first scale assessing the severity of ulcerative colitis episodes, which is still being used today, was developed in 1955 by Truelove and Witts while they were studying the influence of cortisone on the course of ulcerative colitis. Currently, new scales are still being developed which aim to facilitate and standardise therapeutic decisions of doctors taking care of patients with ulcerative colitis as far as possible. Due to the prevalence of the disease the patients do not always find themselves in referral centres, but also in smaller hospitals that do not specialise in ulcerative colitis therapy. From the point of view of a surgeon the most important issue is determining whether the patient requires surgery or whether pharmacotherapy alone is sufficient. A rapid course of the disease, a high severity of complaints and malnutrition often leading to cachexia precludes conservative treatment and requires more radical management of the disease and surgical intervention.
Introduction
Ulcerative colitis, alongside Crohn’s disease and indeterminate colitis, is an inflammatory bowel disease. It is characterised by multifactor, still not fully determined aetiology and a chronic course with periods of remissions and exacerbations. Ulcerative colitis (UC) is most common in the Caucasian population: primarily in Europe and North America. The incidence of ulcerative colitis in Europe is approximately 10/100,000 (1); a similar incidence of the disease is observed in Great Britain (2). The highest incidence of the disease is observed between 20 and 40 years of age; however, the disease can occur both in children and elderly individuals. Women and men are affected by UC at a similar rate, unlike Crohn’s disease, which affects women more frequently (3). Inflammatory bowel diseases are more common in individuals living in the city and performing office work (4). The first and most characteristic symptom is bloody diarrhoea. It may be accompanied by other manifestations such as the presence of mucus in the stool, abdominal cramps which exacerbate directly before and subside after bowel movement, fever, urgency to defecate, nocturnal bowel movements or even lower gastrointestinal tract bleeding. Severe diarrhoea that occurs during an episode of exacerbation is often the cause of cachexia and dehydration.
Aetiology and pathogenesis
The aetiology of the disease remains unknown, although genetic, immunological and environmental factors have been proven to have a significant impact on the pathogenesis of the disease.
Ulcerative colitis is a hereditary condition in 6-7% of patients. The risk of the disease in first-degree relatives is approximately 5%. The presence of the disease in one’s siblings is associated with a 4.6 times higher risk of the development of UC, while the risk for monozygotic twins is increased as many as 95 times (5). Some of the genes which may increase an individual’s susceptibility to ulcerative colitis include: RNF186, OTUD3, PLA2G2E, IFNG, IL26, IL22 (6).
The immune system plays a very important role in the pathophysiology of UC. An autoimmune nature of the disease has been proven: in the course of the disease antibodies are produced which attack the body’s own cells. The main role in the disease is played by Th2 cells in that they prevent the inhibition of the inflammatory response and by IL-7, which is responsible for the proliferation and differentiation of T cells (7).
A distinct environmental factor which affects the chances and course of the disease is nicotine addiction: tobacco smoking has a protective effect, reduces the risk of extensive ulcerative colitis and limits extracolonic complications. Salmonella or Campylobacter infection is associated with an 8-10-time higher risk of the development of ulcerative colitis (8). Among medicines which increase the chances of the disease non-steroidal anti-inflammatory drugs (NSAIDs) play the most important role, which significantly increase the risk of exacerbation. A protective effect of appendectomy and its mitigating influence on the course of the disease are being investigated (9). Diet-related factors also affect the risk of the UC: a diet rich in sugar, fat and meat is conducive to the development of the disease, while a vegetable-rich diet reduces the risk of an inflammatory bowel disease over the course of the whole lifespan.
Clinical scales
One of the first symptoms which may raise the suspicion of ulcerative colitis is most often diarrhoea (lasting usually over 6 weeks) with traces of blood, therefore, it is the most important parameter in every scale assessing disease activity. The number of bowel movements in the course of the disease can be as many as 20 daily.
In 2005, during the World Congress of Gastroenterology in Montreal a classification of UC based on the extensiveness of lesions in the colon was proposed. This aspect of the disease was intended to be key to the choice of local vs. systemic treatment (10).
This scale differentiates between:
– E1: proctitis, in which the disease is limited to the rectum (the proximal extent of inflammation is distal to the rectosigmoid junction). In this form of the disease the patients mainly complain of urgency to defecate, lower gastrointestinal tract bleeding and sometimes constipation,
– E2: distal/left-sided UC, in which the disease is limited to a proportion of the colorectum distal to the splenic flexure,
– E3: extensive UC, in which the disease extends proximally to the splenic flexure, sometimes involving even the whole colon (pancolitis) and sometimes reaching the distal part of the ileum.
During the same congress a classification of the severity of relapse was also agreed upon. It includes the following:
– S0: clinical remission; asymptomatic,
– S1: mild UC; signs and symptoms: up to 4 bowel movements daily (with or without blood); no systemic manifestations; normal ESR,
– S2: moderate UC; signs and symptoms: > 4 bowel movements daily; moderate systemic manifestations,
– S3: severe UC; signs and symptoms: > 6 bowel movements with blood daily; HR: > 90/min; body temperature: ≥ 37.5°C; Hb < 10.5 g%; ESR ≥ 30 mm/h.
Another classification of ulcerative colitis, also known as the Clinical Activity Index (CAI), was proposed by Rachmilewitz (tab. 1). This classification is used for the assessment of disease activity based on such data as: the number of bowel movements a week, blood in the stool, the general condition of the patient, abdominal pain, blood temperature, extracolonic symptoms, ESR and Hb levels (12).
Tab. 1. Clinical Activity Index for ulcerative colitis by Rachmilewitz
| Number of stools weekly |
| < 18 | 0 |
| 18-35 | 1 |
| 36-60 | 2 |
| > 60 | 3 |
| Presence of blood in stool on average |
| none | 0 |
| a small amount; < 30% of bloody stools | 2 |
| a large amount; > 30% of bloody stools | 4 |
| General condition |
| good | 0 |
| average | 1 |
| poor | 2 |
| very poor | 3 |
| Abdominal pain |
| none | 0 |
| mild | 1 |
| moderate | 2 |
| severe | 3 |
| Temperature |
| 37-38°C | 0 |
| > 38°C | 3 |
| Extraintestinal manifestations |
| iritis | 3 |
| erythema nodosum | 3 |
| arthritis | 3 |
| Laboratory findings |
| ESR > 50 mm/h | 1 |
| ESR > 100 mm/h | 2 |
| Hb < 10 g% | 4 |
Extraintestinal manifestations are found only in approximately 30% of UC patients (11). Some of them are associated with disease exacerbation, such as, for instance, peripheral arthritis or erythema nodosum. There are also additional manifestations occurring irrespectively of the phase of the disease: pyoderma gangrenosum, primary sclerosing cholangitis (PSC), peripheral arthritis, ankylosing spondylitis (AS), psoriasis, uveitis, conjunctivitis. The scale takes into account three most common extraintestinal manifestations. A patient who complains only of gastrointestinal symptoms can receive 22 out of 31 points at the peak of the disease, since 9 points refer to extraintestinal manifestations.
Another scale that is also used to assess disease activity is a system developed by Powell-Tuck in 1978 (tab. 2), which is also called St. Mark’s Index. There are 10 factors to be assessed, which include clinical manifestations and biochemical markers as well as the appearance of the intestinal mucosa on colonoscopy (13).
Tab. 2. Powell-Tuck Index
| | Score |
| Factor | 0 | 1 | 2 | 3 |
| Bowel frequency in 24 h | < 3 | 3-6 | > 6 | |
| Stool consistency | Formed | Semi-formed | Liquid | |
| Abdominal pain | None | Associated with bowel motions | Chronic | |
| Anorexia | None | Present | | |
| Nausea and vomiting | None | Present | | |
| General health | Normal | Slightly impaired, but the patient is able to lead an active life | Activities restricted | Unable to work |
| Bleeding | No signs | Trace | More than trace | |
| Abdominal tenderness | None | Mild | Marked | Rebound tenderness |
| Extracolonic manifestations | None | Mild | Severe | Involving multiple systems |
| Temperature (°C) | < 37.1 | 37.1-38 | > 38 | |
| Appearance on colonoscopy | Non-haemorrhagic lesions | Contact bleeding, but no spontaneous bleeding | Spontaneous bleeding | |
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Piśmiennictwo
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