Ulcerative colitis may present at any age. Men and women are equally affected. In adults at presentation about 40 to 50% have proctitis, 30% to 40% left sided colitis, and 20% extensive colitis or pancolitis.1 In children, only 25% present with proctitis alone, 30% have left sided colitis, and in 45% the disease extends to the transverse colon or beyond.

Table 1 lists typical symptoms at presentation.2 Virtually all patients with ulcerative colitis have rectal bleeding or bloody diarrhoea. Delays in presentation are common through such diverse reasons as fear of cancer or a general reluctance to discuss matters relating to bowel habit (Table 2). Many patients may complain of pain of colonic origin, often left sided and related to defaecation. There are no specific clinical signs on general examination, but inflammation of the rectal mucosa (proctitis) can readily be seen at proctoscopy.

Rectal bleeding
The most common symptom of ulcerative colitis is rectal bleeding. Patients with haemorrhagic proctitis usually complain of passing fresh blood either separately from the stool or streaked on the surface of the normal or hard stool.3 When the disease extends beyond the rectum, blood is usually mixed with stool or there may be grossly bloody diarrhoea. When the disease is severe, patients pass liquid stool containing blood, pus, and faecal matter.

Diarrhoea
Diarrhoea is not always present in patients with ulcerative colitis. Constipation occurring as presenting complaint is most often associated with disease limited to the rectum and recto sigmoid, presumably because spasm prevents faeces from entering the involved area.3 However most patients with active disease complain of diarrhoea and may have nocturnal diarrhoea. Postprandial diarrhoea is common. Urgency with a feeling of incomplete evacuation is common in proctitis. The diarrhoea is often associated with passing large quantities of mucus, often with blood and pus. Urgency and tenesmus, which are common symptoms when the rectum is inflamed, are caused by poor compliance and loss of the reservoir capacity of the inflamed rectum.⁴ With severe inflammation; the urgency can be sufficiently acute as to cause incontinence.

Abdominal pain
Abdominal pain is a common complaint, although it is minimal or absent in acute mild attacks or in patients who have an insidious onset. The pain is usually colicky or crampy in character and most often confined to the left lower abdominal quadrant or hypogastrium. Characteristically, the pain occurs in association with the desire to defaecate and is relieved by bowel evacuation. For most patients with ulcerative colitis, pain is not a prominent symptom. Some patients with active disease may experience vague lower abdominal discomfort, an ache in the left iliac fossa, or mild central abdominal cramping. Severe cramping and abdominal pain can occur in association with severe attacks of the disease.

Other symptoms
Disease of moderate to severe activity may often be associated with systemic symptoms like anorexia, nausea, and vomiting. Also some patients may have profound weight loss and hypoalbuminaemia. Patients also may complain of the symptoms of anaemia.

Signs
Patients with mild to moderately severe disease exhibit few abnormal physical signs. The affected portion of the colon may be tender on abdominal palpation. Digital examination of the rectum may reveal oedematous and velvety mucosa. Patients with severe attacks look ill, with evidence of weight loss and depletion of salt and water. The abdomen may be distended and tympanic, the colon tender, and the bowel sounds reduced. Signs of extraintestinal manifestations may be present.

Clinical assessment of disease severity
Clinical severity is assessed by criteria of Truelove and Witt’s.⁵
Mild - fewer than four stools daily, with or without blood, with no systemic disturbances and a normal erythrocyte sedimentation rate (ESR)
Moderate - More than four stools daily but with minimal systemic disturbance
Severe - More than six stools daily with blood and with evidence of systemic disturbance, as shown by fever, tachycardia, anaemia, or an ESR greater than 30.

Extraintestinal manifestations
Extraintestinal manifestations are commonly present in patients with ulcerative colitis6 and can be classified as shown in Table 2. The manifestations that are related to the activity of the colitis usually settle when the colonic inflammation is brought into remission.

Skin
The most common rashes are related to therapy and include hypersensitivity rashes to sulphasalazine and urticarial reactions to mesalamine.

Erythema nodosum occurs in 2% to 4% of patients with active ulcerative colitis.

Erythema nodosum presents as multiple tender and inflamed nodules, mostly on the anterior aspect of the lower legs.

Pyoderma gangrenosum is rare and occurs in 1% to 2% of patients. It is usually related to active colonic inflammation. The lesions are usually multiple and may occur on the trunk or the limbs.

Mouth
Crops of oral aphthous ulcers occur in at least 10% of patients with active ulcerative colitis and rapidly resolve once the disease goes into remission.

Eyes
Episcleritis or anterior uveitis occurs in 5% to 8% of patients with active colitis. Mild conjunctivitis is also common. Topical glucocorticoids are useful for controlling symptoms.

Joints
An acute arthropathy occurs in 5% to 10% of patients with an acute attack of ulcerative colitis.7 The arthropathy is asymmetric, affects large joints and is nonerosive. It resolves as the colitis goes into remission. Asymmetric, small joint arthropathy, unrelated to activity of colitis occurs in 5% of patients.7 Sacroiliitis occurs in 5% of patients. Ankylosing spondylitis affects 1% to 2% of patients, and approximately 60% of these have HLA-B27 phenotype. The natural history of spondylitis is independent of the ulcerative colitis. Spondylitis should be treated with nonsteroidal anti-inflammatory drugs and physiotherapy.

Liver disease
The major liver complication of ulcerative colitis is primary sclerosing cholangitis (PSC), which occurs in approximately 3% of all patients. It is diagnosed by endoscopic or magnetic resonance cholangiography. Both intrahepatic and extrahepatic ducts may be involved, leading to the characteristic radiological features of beading, irregularity, and stricturing of the ducts. Most patients with sclerosing cholangitis have total colitis, which is often mild. The colitis may go undetected in patients presenting primarily with liver disease unless sigmoidoscopy and rectal biopsy are performed. The liver disease is progressive and independent of the outcome of the colitis.

There is no satisfactory treatment for sclerosing cholangitis, although ursodeoxycholic acid may delay disease progression. Glucocorticoids may benefit few patients with active liver cell damage. For patients with end-stage liver disease, liver transplantation should be considered and can be highly successful.⁸

Thromboembolism
Deep vein thromboses and pulmonary emboli are well-recognized complications of ulcerative colitis. Patients with these complications should be treated with anticoagulants.

References

1. Whitehead R. Ulcerative colitis. In Whitehead R (ed) : Gastrointestinal and Oesophageal Pathology. Edinburgh, Churchill Livingstone, 1989; p. 522.
2. Both H, Torp-Pedersen K, Kreiner S, et al. Clinical appearance at diagnosis of ulcerative colitis and Crohn’s disease in a regional patient group. Scand J Gastroenterol 1983; 18 (7) : 987.
3. Rao SS, Holdsworth CD, Read NW. Symptoms and stool patterns in patients with ulcerative colitis. Gut 1988; 29 (3) : 342.
4. Rao SS, Read NW, Davison PA, et al. Anorectal sensitivity and responses to rectal distension in patients with ulcerative colitis. Gastroenterology 1987; 93 (6) : 1270.
5. Truelove SC, Witts LJ. Cortisone in ulcerative colitis - final report on a therapeutic trial. BMJ 1955; 2 : 1041.
6. Snook J, Jewell DP. Management of the extra-intestinal manifestations of ulcerative colitis and Crohn’s disease. Semin Gastrointest Dis 1991; 2 : 115.
7. Orchard TR, Wordsworth BP, Jewel DP. Peripheral arthropathies in inflammatory bowel disease : Their articular distribution and natural history. Gut 1998; 42 (3) : 387.
8. Narumi S, Roberts JP, Emond JC, et al. Liver transplantation for sclerosing cholangitis. Hepatology 1995; 22 (2) : 451.