presentation UC

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بسم الله الرحمن الرحيم

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Ulcerative colitis Dr M Nasir Khan

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Ulcerative colitis (UC) is a relapsing, remitting inflammatory disease of the colonic mucosa and submucosa .

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The prevalence of UC in the United States is 150-200/100,000 of population. A genetic contribution to the disease is indicated by the increased incidence of UC (of 30 to 100 times that of the general poupulation ) among first-degree relative of patients with UC. Approximately 5,300 children hospitalized in 1997 in the US ( Guthery et al, J Peds 2003) Approximately 1,000,000 in the US have the disorder.

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The characteristic pathology is one of chronic inflammation characterized by large numbers of lymphocytes and histiocytes in the diseased mucosa and submucosa with an acute inflammatory infiltrate composed of neutrophils variably present.

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UC: is a form of (IBD). It is a form of colitis, of that includes characteristic ulcers, or open sores, in the colon. The main symptom of active disease is usually diarrhea mixed with blood, of gradual onset. UC is, however, a systemic disease that affects many parts of the body outside the intestine. Because of the name, IBD is often confused with irritable bowel syndrome ("IBS"), a troublesome, but much less serious condition.

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UC is an intermittent disease, with periods of exacerbated symptoms, and periods that are relatively symptom-free. Although the symptoms of UC can sometimes diminish on their own, the disease usually requires treatment to go into remission.

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Although UC has no known cause, there is a presumed genetic component to susceptibility. The disease may be triggered in a susceptible person by environmental factors. Although dietary modification may reduce the discomfort of a person with the disease, UC is not thought to be caused by dietary factors. Although UC is treated as though it were an autoimmune disease, there is no consensus that it is such.

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UC is a systemic disease that affects many parts of the body. Sometimes the extra-intestinal manifestations of the disease are the initial signs, such as painful, arthritic knees in a teenager. It is, however, unlikely that the disease will be correctly diagnosed until the onset of the intestinal manifestations .

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The clinical presentation of UC depends on the extent of the disease process. Patients usually present with diarrhea mixed with blood and mucus, of gradual onset. They also may have signs of weight loss, and blood on rectal examination. The disease is usually accompanied with different degrees of abdominal pain, from mild discomfort to severely painful cramps.

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Extent of involvement UC is normally continuous from the rectum up the colon. The disease is classified by the extent of involvement, depending on how far up the colon the disease extends: Distal colitis, potentially treatable with enemas: Proctitis : Involvement limited to the rectum. Proctosigmoiditis : Involvement of the rectosigmoid colon, the portion of the colon adjacent to the rectum. Left-sided colitis: Involvement of the descending colon, which runs along the patient's left side, up to the splenic flexure and the beginning of the transverse colon.

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Extensive colitis , inflammation extending beyond the reach of enemas: Pancolitis : Involvement of the entire colon, extending from the rectum to the cecum , beyond which the small intestine begins.

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Severity of disease In addition to the extent of involvement, UC patients may also be characterized by the severity of their disease. Mild disease correlates with fewer than four stools daily, with or without blood, no systemic signs of toxicity, and a normal erythrocyte sedimentation rate (ESR). There may be mild abdominal pain or cramping. Patients may believe they are constipated when in fact they are experiencing tenesmus , which is a constant feeling of the need to empty the bowel accompanied by involuntary straining efforts, pain, and cramping with little or no fecal output. Rectal pain is uncommon.

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Moderate disease correlates with more than four stools daily, but with minimal signs of toxicity. Patients may display anemia (not requiring transfusions), moderate abdominal pain, and low grade fever, 38 to 39 °C Severe disease, correlates with more than six bloody stools a day, and evidence of toxicity as demonstrated by fever, tachycardia, anemia or an elevated ESR.

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Fulminant disease correlates with more than ten bowel movements daily, continuous bleeding, toxicity, abdominal tenderness and distension, blood transfusion requirement and colonic dilation. Patients in this category may have severe inflammation extending beyond just the mucosal layer, causing impaired colonic motility and leading to toxic megacolon . If the serous membrane is involved, colonic perforation may ensue. Unless treated, fulminant disease will soon lead to death.

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Extraintestinal features As UC is a systemic disease, patients may present with symptoms and complications outside the colon. These include the following: aphthous ulcers of the mouth . Ophthalmic . Iritis or uveit . Episcleritis .

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Patients with ulcerative colitis can occasionally have aphthous ulcers involving the tongue, lips, palate and pharynx

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Musculoskeletal: Seronegative arthritis, which can be a large-joint oligoa rthritis (affecting one or two joints), or may affect many small joints of the hands and feet Ankylosing spondylitis , arthritis of the spine Sacroiliitis , arthritis of the lower spine Cutaneous Erythemanodosum , which is a panniculitis , or inflammation of subcutaneous tissue involving the lower extremities Pyoderma gangrenosum , which is a painful ulcerating lesion involving the skin

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Deep venous thrombosis and pulmonary embolism Autoimmune hemolytic anemia clubbing , Primary sclerosing cholangitis , or inflammation of the bile ducts

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Similar conditions The following conditions may present in a similar manner and should be excluded: Crohn's disease Infectious colitis , which is typically detected on stool cultures Pseudom embranous colitis, or Clostridium difficile-ssociated colitis, bacterial upsets often seen following administration of antibiotics Ischemic colitis, inadequate blood supply to the intestine, which typically affects the elderly Radiation colitis in patients with previous pelvic radiotherapy Chemical colitis resulting from introduction of harsh chemicals into the colon from an enema or other procedure

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Comparison to Crohn's Disease The most common disease that mimics the symptoms of UC is Crohn's disease, as both are IBD that can affect the colon with similar symptoms. It is important to differentiate these diseases, since the course of the diseases and treatments may be different. In some cases, however, it may not be possible to tell the difference, in which case the disease is classified as indeterminate colitis.

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Endoscopy The best test for diagnosis of UC remains endoscopy. Full colonoscopy to the cecum and entry into the terminal ileum is attempted only if diagnosis of UC is unclear. Otherwise, a flexible sigmoidoscopy is sufficient to support the diagnosis. The physician may elect to limit the extent of the exam if severe colitis is encountered to minimize the risk of perforation of the colon. Endoscopic findings in UC include the following: Loss of the vascular appearance of the colon, Erythema (or redness of the mucosa) and friability of the mucosa Superficial ulceration, which may be confluent, and Pseudopolyps .

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UC is usually continuous from the rectum, with the rectum almost universally being involved. There is rarely peri -anal disease, but cases have been reported. The degree of involvement endoscopically ranges from proctitis or inflammation of the rectum, to left sided colitis, to pancolitis , which is inflammation involving the ascending colon

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Endoscopic image of ulcerative colitis affecting the left side of the colon. The image shows confluent superficial ulceration and loss of mucosal architecture. Crohn's disease may be similar in appearance, a fact that can make diagnosing UC a challenge.

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Colonic pseudopolyps of a patient with intractable ulcerative colitis. Colectomy specimen

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Histology Biopsies of the mucosa are taken to definitively diagnose UC and differentiate it from Crohn's diseas , Microbiological samples are typically taken at the time of endoscopy. The pathology in UC typically involves distortion of crypt architecture, inflammation of crypts ( cryptitis ), frank crypt abscesses, and hemorrhage or inflammatory cells in the lamina propria . In cases where the clinical picture is unclear, the histomorphologic analysis often plays a pivotal role in determining the management

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Course and complications Progression or remission Patients with UC usually have an intermittent course, with periods of disease inactivity alternating with "flares" of disease. Patients with proctitis or left-sided colitis usually have a more benign course: only 15% progress proximally with their disease, and up to 20% can have sustained remission in the absence of any therapy. Patients with more extensive disease are less likely to sustain remission, but the rate of remission is independent of the severity of disease.

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UC and colorectal cancer There is a significantly increased risk of colorectal cancer in patients with UC after 10 years if involvement is beyond the splenicflexure . Those with only proctitis or rectosigmoiditis usually have no increased risk. It is recommended that patients have screening colonoscopies with random biopsies to look for dysplasia after eight years of disease

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Primary sclerosing cholangitis (PSC) UC has a significant association with (PSC), a progressive inflammatory disorder of small and large bile ducts. As many as 5% of patients with UC may progress to develop (PSC). Mortality The effect of UC on mortality is unclear, but it is thought that the disease primarily affects quality of life, and not lifespan.

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Treatment Standard treatment for UC depends on extent of involvement and disease severity . The goal is to induce remission initially with medications, followed by the administration of maintenance medications to prevent a relapse of the disease. The concept of induction of remission and maintenance of remission is very important.

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The medications used to induce and maintain a remission somewhat overlap, but the treatments are different. Physicians first direct treatment to inducing a remission which involves relief of symptoms and mucosal healing of the lining of the colon and then longer term treatment to maintan the remission. Current treatments have been effective for many patients with UC but have numerous limitations for patients with modeate to severe disease.

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Drugs used Aminosalicylates are the mainstay of UC pharmacotherapy for induction and maintenance of remission for patietns with mild to moderate disease. Sulfasalazine has been a major agent in the therapy of mild to moderate UC for over 50 years. Mesalazine , also known as 5-aminosalicylic acid Balsalazide - Disodium , also known as Colazal . Olsalazine , also known as Dipentum . Corticosteroids : often required for the one-third of patients who fail to respond to 5-ASAs Immunosupressive drugs: have a role in maintenance of remission in moderate to severe UC

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Biological treatment management of cancer, autoimmune diseases, and diseases of unknown cause that result in symptoms due to immune related mechanisms .( Infliximab , Visilizumab ). Surgery UC can generally be cured by surgical removal of the large intestine. This procedure is necessary in the event of: exsanguinating hemorrhage, frank perforation or documented or strongly suspected carcinoma. Surgery is also indicated for patients with severe colitis or toxic megacolon . Patients with symptoms that are disabling and do not respond to drugs may wish to consider whether surgery would improve the quality of life. In rare cases the extra-intestinal manifestations of the disease may require removal of the colon.

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Therapeutic Pyramid for Active UC Severe Moderate Mild Systemic Corticosteroids Aminosalicylates Surgery Oral Steroids AZA/6-MP Cyclosporine Infliximab

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Alternative treatments Smoking : Unlike Crohn's disease, UC has a lesser prevalence in smokers than non-smokers . Dietary modification : Dietary modification may reduce the symptoms of the disease. Lactose intolerance is noted in many ulcerative colitis patients. Many dietary approaches have purported to treat UC, including the Elaine Gottschall's specific carbohydrate diet and the "anti-fungal diet" (Holland/Kaufmann ).The use of elemental and semi-elemental formula has been successful in pediatric patients. Bacterial recolonization Probiotics may have benefit. And promise for people with UC.

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Intestinal parasites IBD is less common in the developing world. Some have suggested that this may be because intestinal parasites are more common in underdeveloped countries. Some parasites are able to reduce the immune response of the intestine, an adaptation that helps the parasite colonize the intestine. The decrease in immune response could reduce or eliminate the IBD .

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