Ulcerative Colitis

Ulcerative colitis (UC) is inflammation of the lining of the colon. UC is one of the major forms of inflammatory bowel disease (IBD). It is sometimes hard to determine whether a person has UC or Crohn’s disease, another form of IBD.

The colon, or large intestine, makes up the last few feet of the digestive tract. The colon includes the rectum, where stool is stored before a bowel movement. Occasionally, people with UC also can have irritation in the stomach or the small intestine.

“Typical” UC describes inflammation starting anywhere in the colon and then spreading evenly over the entire intestine lining, all the way through the rectum. “Atypical” UC may describe inflammation of the colon that is patchy, does not include the rectum, or involves the stomach. UC also can be classified by its severity, with “acute severe colitis” extending deeper into tissues of the colon and causing symptoms that might require hospitalization.

Some systems classify UC by location. In ulcerative proctitis, only the rectum is inflamed. In left-sided colitis, the inflammation includes the part of the colon on the left side of the abdomen. In extensive colitis, the inflammation can include the part of the colon that crosses the upper abdomen and even the part that extends from the bottom to the top of the right abdomen. Over time, a person’s inflammation can change from one of these categories to another.

Both genetic and environmental factors likely interact to cause UC, which is becoming more common worldwide. In the United States, about 3.1 million children and adults have either Crohn’s disease or UC. UC can occur at any age but often starts during adolescence rather than in earlier childhood. It rarely is seen in infants.

All people have billions of bacteria and other microorganisms in their intestines. Researchers think that UC occurs when the immune system has an excessive or abnormal response to some of these microorganisms. For that reason, UC is categorized as an “autoimmune” disease. Influences on the intestinal microorganisms and influences on the immune system are probably both involved in causing UC. These influences include genetic as well as environmental factors.

• Genetic factors: A family history of IBD or other autoimmune conditions is a risk factor for UC. UC can occur in people of any ethnic group or national background. However, it is more common in certain groups, including people of Ashkenazi Jewish heritage. There is no single gene or mutation that causes UC. However, some of the genes associated with UC are involved in the immune system.
• Environmental factors: We still have limited information about the environmental causes of UC, and whether they are the same for children and adults. However, there generally appear to be both high-risk and protective factors.
  • High-risk factors:
    • Living in a northern country (such as the United States, Canada, or northern Europe)
    • Use of antibiotics in early childhood
    • Past use of acid blockers
    • High intake of red meat, fat, and sugar
  • Protective factors:
    • Breastfeeding as an infant
    • High intake of fruits, vegetables, and dietary fiber
    • Surgery to remove the appendix (appendectomy)

People with UC may have some or all of these symptoms:

• Abdominal pain: The pain may be in the lower left part of the abdomen or more generalized around the abdomen. This pain may be persistent, severe, and could possibly wake your child from sleep.
• Diarrhea: Passage of watery stool or frequent stool that is different from your child’s normal pattern. It could be mild to severe and may frequently wake your child from sleep.
• Tenesmus: This is the sensation of fullness in the rectum, which may lead a person to feel that they still need to use the toilet even after they have passed a bowel movement.
• Blood in the stool
• Weight loss or lack of weight gain
• Poor growth, or not gaining height as quickly as expected
• Unexplained fevers
• Joint pain
• Skin changes, which may look like sores, bruises, or large hives on the shins
• Constipation: Although not typical, some people with UC have constipation, although this is usually in combination with other symptoms.

Other symptoms, referred to as extra-intestinal manifestations, occur outside the digestive tract. These are detailed in the “complications” section below.

Your primary care physician will perform an initial evaluation if you are concerned that your child has UC. Ask your doctor for referral to a pediatric gastroenterologist if your child has blood in the stool, persistent diarrhea, persistent abdominal pain, weight loss, or slow growth.

• Blood: Your doctor may collect blood to test for inflammatory markers, such as erythrocyte (red blood cell) sedimentation rates or C-reactive protein, as well as a complete blood count to test for high platelet levels (called thrombocytosis) or low red blood cells (called anemia), or a comprehensive metabolic panel to test for a variety of issues including low protein levels (called hypoalbuminemia) or abnormal electrolytes. Blood tests can be normal even if a patient has UC. Once a diagnosis of IBD is made, levels of certain antibodies in the blood are sometimes helpful in determining if a patient has UC or Crohn’s disease. However, these are not reliable for an initial diagnosis.
• Stool: Stool tests may be done to rule out infection in the gut. Markers of inflammation that can be identified in the stool (calprotectin or lactoferrin) may be tested.
• Endoscopy and colonoscopy: These are the main tests to diagnose UC. Esophagogastroduodenoscopy (EGD) uses an endoscope, or tube with a camera, to inspect the upper digestive tract, including the esophagus, stomach, and part of the intestines. Colonoscopy uses an endoscope to inspect the colon and small intestine. Your child will need a clean-out (taking laxatives to empty the digestive tract of stool) before a colonoscopy. Tiny samples of the intestine, called biopsies, are taken to look at under a microscope and confirm the diagnosis.
• Imaging: The following imaging tests may be used to help diagnose UC and/or rule out Crohn’s disease:
  • Contrast studies, upper gastrointestinal series with small bowel follow-through
  • Abdominal computed tomography (CT) scan
  • Magnetic resonance enterography (MRE): This may be performed instead of a CT scan because CT scans expose the patient to radiation.
  • Capsule endoscopy: This is a swallowed pill camera that takes pictures within parts of the small intestine that cannot be reached by an endoscope. If this test is done, your child may need to first swallow a “patency capsule” to ensure that the capsule endoscopy is safe. A clean-out (taking laxatives to empty the digestive tract of stool) is needed in advance.

The goal of treatment in UC is for the patient to feel entirely well, with no ongoing inflammation and no diarrhea, bleeding, or pain. Other goals include minimizing the number of “flares,” or recurrent episodes of inflammation, and making sure your child is growing appropriately.

To reach these goals, your doctor will discuss different therapies for UC depending on the severity and location of your child’s inflammation. Your doctor may mention treatments to induce remission (to make the symptoms disappear) or for maintenance of remission (to prevent the symptoms from returning). UC treatment can include a combination of:

• Medication
• Nutritional and complementary therapy
• Surgery

Your doctor also may recommend additional vaccinations and other measures to prevent or treat UC complications or associated conditions. You might receive referrals to see an ophthalmologist (eye specialist), dermatologist (skin specialist), rheumatologist (autoimmune specialist), surgeon, dietician, psychotherapist, or other medical professional.

Therapy for UC includes the following medications. Each medication has its own risks and benefits and may not be appropriate for your child based on the severity of inflammation and other factors. Your gastroenterologist should review both the effectiveness and potential side effects in detail with you and your child.

In addition to the treatments listed below, your child also might need antibiotics if there is a concern for infection that might make the UC symptoms worse. It is common for people with UC to use multiple different medications before they find the regimen that is most effective or to change medications over time when a treatment is no longer working. New medications also are being studied and may become available over the next few years.

• Corticosteroids (“steroids”): prednisone, prednisolone, methylprednisolone, budesonide - These medications are used for a short time (a few weeks to a few months) to decrease or reverse inflammation and to control symptoms. These medications are never stopped abruptly. Your doctor will tell you how to gradually reduce the dose to stop treatment. Because the body breaks down and processes different steroid medications differently, some have significant side effects while others do not. Based on the location and severity of inflammation, they might be given intravenously (IV), orally, or rectally with an enema or suppository.
• Aminosalicylates: sulfasalazine, mesalamine (brand names: Pentasa®, Asacol®, Lialda®, Apriso®), olsalazine, balsalazide - These are among the safest medications used to treat UC but may not be effective on their own if inflammation is severe. These medications may have some side effects but can be used long-term as maintenance treatment. Based on the location of inflammation, they might be given orally, or rectally with an enema or suppository.
• Immunomodulators: azathioprine (AZA) and 6-mercaptopurine (6MP) - These medications are primarily used as maintenance treatment, to prevent the recurrence of inflammation, or while steroids are being decreased and discontinued. They are also often used with other medications, such as biologics or aminosalicylates. These medications typically take 3–6 months to be effective. These are given orally.
• Immunomodulators: methotrexate - Methotrexate is another immunomodulatory medicine used as a maintenance treatment, often in combination with another medication. This medicine typically takes 4 weeks to be effective. It can be given orally or by weekly injection.
• Anti-tumor necrosis factor (TNF) agents or TNF inhibitors: infliximab (brand name: Remicade®), adalimumab (brand name: Humira®), golimumab (brand name: Simponi®) - Anti-TNF agents are usually used in patients with moderate to severe IBD and are among the most effective treatments. Some are given by IV and some by injection. Because these medications can cause some suppression of the immune system, patients are checked for tuberculosis exposure before starting the medications. Of note, golimumab has been formally approved by the FDA for use in adults, but not children, with UC. However, it may be prescribed in patients younger than 18 years of age based on the patient’s previous reactions to infliximab and adalimumab.
• Anti-integrin agents: vedolizumab (brand name: Entyvio®) - Vedolizumab has been formally approved by the FDA for use in adults, but not children, with UC. However, it is commonly prescribed in patients younger than 18 years of age who have UC and do not respond to other medications. These medicines frequently take a longer time (months) to have an effect. Your doctor may sometimes add other medications during this waiting period. Vedolizumab is given by IV.
• Anti-interleukin agents: ustekinumab (brand name: Stelara®) - Similar to vedolizumab, ustekinumab has been formally approved by the FDA for use in adults, but not children, with UC. However, it is commonly prescribed in patients younger than 18 years of age who have UC and do not respond to other medications. Anti-interleukin agents are usually used in Crohn’s disease patients who do not respond to corticosteroids, immunomodulators, and anti-TNF agents. Ustekinumab is given by injection.
• JAK inhibitors: tofacitinib (brand name: Xeljanz®) - Similar to vedolizumab and ustekinumab, tofacitinib has been formally approved by the FDA for use in adults, but not children, with UC. It is sometimes used in children or adolescents who have not responded or have stopped responding to all the other treatments listed above. It is given orally.
• Biosimilars: Biosimilars are biologics that are very similar to the original medication.  They are not exact, but extremely close, so they aren’t called generics.  Currently, biosimilars exist for Infliximab and soon adalimumab will have biosimilars.  Insurance companies may request a switch to a biosmilar and this should be discussed with your healthcare provider.  More information on Biosimilars can be found here.

UC is not as responsive to dietary changes as Crohn’s disease. Therefore, nutritional therapy is not yet used as a primary treatment without medicines. However, diet and nutrition are extremely important in the management of UC. Good nutrition is important for healing and well-being, as well as for the reversal of low weight, slow growth, anemia, and vitamin and mineral deficiencies.

Additionally, many people with UC find that their symptoms worsen by eating “trigger foods,” which are not universal but can differ from person to person. Many people also find that foods they tolerate when in remission seem to bother them when they have recurrence of inflammation or a “flare.”

General recommendations for diet and nutrition in patients with UC include:

• Limit red meat or processed meat consumption to once per week or less.
• Limit intake of saturated fats and trans-fats.
• Eat plenty of fruits and vegetables, unless otherwise advised by your medical team. There is no need to avoid fiber.

If the diet is not high enough in micronutrients, it may be helpful to take vitamin and mineral supplements, such as iron, calcium, and vitamin D.

Some people with UC follow restrictive eating plans such as the Specific Carbohydrate Diet or the Crohn’s Disease Exclusion Diet. These diets have not yet been proven in studies to help patients with UC. If you are considering this kind of diet, talk to your doctor and consult with a registered dietician to make sure that your child is getting balanced nutrition.

Alternative therapy is treatment for UC that is not traditional medical therapy. Complementary therapies are used in combination with traditional medicine. 

Some research has been done on complementary and alternative therapies in people with UC. However, there is not enough research to determine if these therapies work. Discuss these options with your child’s gastroenterologist before starting any complementary/alternative therapies.

• Fecal microbiota transplant (FMT): The goal of FMT is to introduce bacteria from the intestine of a healthy donor into the intestine of a patient with UC, to decrease inflammation triggered by the patient’s original bacteria. Researchers are studying the effectiveness, risks, and best techniques for this treatment. Until that information is available, FMT is only recommended as part of medical studies (clinical trials). 
• Herbal supplements: There are many supplements that have been studied in patients with UC, including curcumin, Indigo naturalis, Boswellia serrata (frankincense), Andrographis paniculate (Indian echinacea), wheatgrass juice, aloe vera, myrrh, and chamomile. We do not yet have enough information about their effectiveness to recommend these supplements. Please note that herbal supplements are not regulated by the FDA.
• Probiotics: Probiotics are bacteria and/or yeasts intended to help create a healthy environment in the intestine. Because there are so many kinds of probiotics, it is not possible to make general recommendations about their use. However, in people with UC who have already had surgery to remove their colon, the multi-strain product Visbiome® (also called VSL #3®) can help prevent inflammation from recurring in the remaining intestinal tissue. Visbiome® and E. coli Nissle 1917 (Mutaflor®) also may help induce and maintain remission in patients with mild to moderate UC, although there is more information on its use in adults than in children. Please note that probiotics are not regulated by the FDA.
• Fish oil: Fish oil contains omega-3 fatty acids, which are essential to the human diet, along with omega-6 and omega-9. Omega-3 fatty acids may help prevent or decrease inflammation, including colonic inflammation. These fatty acids are found in foods such as fatty fish (salmon, herring, and mackerel) and some green vegetables.

In ulcerative colitis, the inflamed portion of bowel is limited to the colon (large intestine). Therefore, removal of the entire colon can be considered a cure for the disease.

However, removal of the entire colon may be associated with complications and commonly requires certain lifestyle adjustments. The type of surgery performed depends on the exact reasons for the surgery and should be tailored individually for each patient.

Emergent/Urgent

• Uncontrollable bleeding
• Unresponsive to medical therapy
• Bowel perforation
• Bowel obstruction
• Toxic megacolon
• Cancer

Elective

• Unresponsive to medical therapy

Proctocolectomy with end-ileostomy:

This involves removal of the large bowel, and the end of the small bowel then sticks out through the skin. The small bowel is attached to a bag on the abdomen to collect stool. The opening of the intestine through the skin surface is called an ostomy.

Total colectomy with ileal pouch anal anastomosis:

This involves removal of the large bowel with creation of a “pouch” (reservoir) out of the small bowel, which is connected to the anus. Creation of the pouch allows for reduced frequency of bowel movements after the operation. This is the preferred surgery for ulcerative colitis when possible. This is typically done in 2–3 stages.

Total colectomy with ileorectal anastomosis:

This involves removal of the large intestine with a straight connection between the end of the small bowel and the anus, without creation of a pouch. Without the pouch, there tends to be more frequent bowel movements for the first 6–12 months after the operation.

Direct complications of UC can include:

• Intestinal blockage and/or tearing
• Intestinal bleeding
• Abnormal narrowing of the colon (stricture)
• Slow growth and weight gain
• In the long term, people with UC have a higher rate of colon cancer

Complications outside of the intestine, also called extra-intestinal manifestations, can include:

• Skin: Unusual skin rashes (may be called erythema nodosum or pyoderma gangrenosum)
• Joints: Pain and swelling of joints; ankylosing spondylitis (lower back pain, spine inflammation)
• Eyes: Inflammation of different parts of the eye (called uveitis, episcleritis, or keratitis)
• Mouth: Oral sores (canker sores), which may range in severity from painless to severe and are usually recurrent
• Bone: Reduced bone mineral density
• Blood: Anemia
• Blood vessels: Increased risk of blood clotting, especially in the setting of active disease and/or after surgery
• Liver: Inflammation of the liver may occur from IBD or from the associated conditions of autoimmune hepatitis and primary sclerosing cholangitis
• Kidney: Increased risk of kidney stones
• Psychological: UC is often associated with depression and/or anxiety, perhaps due to some combination of inflammation, anemia, malnutrition, and the stress of painful or disruptive symptoms
• Sleep: Increased risk of sleep disorders

There also are a wide range of complications that can be caused by different UC treatments, including drug allergies and side effects. It is sometimes difficult to know whether it is the UC itself or the medications that are the cause of a symptom or problem. If you are concerned that your child is experiencing a complication or a side effect, be sure to discuss it with your doctor.

In addition to online resources, there are many community support groups. Ask your doctor or nurse about local community support groups, such as the Power of Two Program. Additional resources include the Crohn’s and Colitis Foundation, IBD Support Foundation, NASPGHAN Foundation, and ImproveCareNow.

Resources can also be found on our IBD Resources page.

Authors: Rebecca Cherry, MD, Laura Bauman, MD, Edwin F. de Zoeten MD, PhD
Editor: Athos Bousvaros, MD
August 2021