What is Crohn's disease?
Crohn's disease is a condition that causes inflammation of the wall of the gut. Any part of the gut can be affected. This can lead to various symptoms (detailed below). Crohn's disease is named after Dr Crohn, the person who first described the disease in the 1930s.
Understanding the gut
The gut (gastrointestinal tract) is the long tube that starts at the mouth and ends at the anus. When we eat, food passes down the oesophagus (gullet), into the stomach and then into the small intestine.
The small intestine has three sections - the duodenum, jejunum and ileum. The small intestine is where food is digested and absorbed into the bloodstream. The structure of the gut then changes to become the large intestine (colon and rectum, sometimes called the large bowel).
The colon absorbs water, and contains food that has not been digested, such as fibre. This is passed into the last part of the large intestine, where it is stored as faeces. Faeces (motions or stools) are then passed out of the anus into the toilet.
Who gets Crohn's disease?
Crohn's disease is diagnosed in about 1 in 10,000 people every year. There are about 115,000 people in the UK currently with this disease. It can develop at any age but most commonly starts between the ages of 15 and 30. It affects women slightly more often than men. If you have a family member with Crohn's disease, you are more likely to develop the condition yourself. It is also more common in people who have had their appendix removed, for the first five years after the operation.
Which part of the gut is affected in Crohn's disease?
In Crohn's disease, one or more patches of inflammation develop in parts of the gut. Any part of the gut can be affected. However, the most common site for the disease first to start is the last part of the small intestine (the ileum). The ileum is affected in about half of cases. Other parts of the small intestine and the colon are also commonly affected. The mouth, oesophagus and stomach are affected much less commonly.
A patch of inflammation may be small, or spread quite a distance along part of the gut. Several patches of inflammation may develop along the gut, with normal sections of gut in between. In about 3 in 10 cases, the inflammation occurs just in the small intestine. In about 2 in 10 cases the inflammation occurs just in the colon. In a number of cases, the inflammation occurs in different places in the gut.
What causes Crohn's disease?
The cause is not known. About 3 in 20 people with Crohn's disease have a close relative who also has it. This means there may be some genetic factor. However, other factors such as a bacterium or virus (germ) may be involved. One theory is that a germ may trigger the immune system to cause inflammation in parts of the gut in people who are genetically prone to develop the disease.
Crohn's disease has become more common in recent years, but the reason for this is not known. It is about twice as common in smokers than average. Also, on average, smokers tend to have more severe disease than non-smokers. The oral contraceptive pill and non-steroidal anti-inflammatory tablets (usually used for joint inflammation) have also been implicated as possible factors in triggering the disease to start.
What are the symptoms during a flare-up of Crohn's disease?
Symptoms are due to inflammation in the wall of the affected parts of the gut. When the disease flares up, the inflammation may cause one or more of the following:
- Diarrhoea is the most common first symptom. It can vary from mild to severe. The diarrhoea may be mixed with mucus, pus or blood. An urgency to get to the toilet is common. A feeling of wanting to go to the toilet but with nothing to pass is also common (tenesmus).
- Pain occurs in about 7 in 10 cases. The site of the pain depends on which part of the gut is affected. The last part of the small intestine (ileum) is the most common site. Therefore, a common area of pain is the lower right side of the abdomen. When Crohn's disease first develops it is sometimes mistaken for appendicitis. The severity of pain can vary from person to person. Also, a sudden change or worsening of pain may indicate a complication (see below).
- Weight loss that is not intentional is another common symptom.
- Ulcers. An ulcer is a raw area of the lining of the gut which may bleed. You may see blood when you pass stools (motions or faeces).
- Generally feeling unwell, which may include loss of appetite, fever, and tiredness.
- Anaemia may occur if you lose a lot of blood.
- Mouth ulcers are common.
- Anal fissures may occur. These are painful cracks in the skin of the anus. Skin tags (small fleshy wart-like lumps) may also appear around the anus.
Symptoms can vary and depend on which part or parts of the gut are affected - for example:
- You may not have diarrhoea if the disease is just in the small intestine.
- A persistent pain in the abdomen without any other symptoms may be due to a small patch of Crohn's disease in the small intestine.
- A severe flare-up can make you generally very ill.
- If large parts of the gut are affected, you may not absorb food well and you may become deficient in vitamins and other nutrients.
Other parts of the body are affected in some people in addition to the gut. These include: inflammation and pain of some joints (arthritis); skin rashes; inflammation of the eye (uveitis); liver inflammation. These problems can cause various symptoms.
It is not clear why these other problems occur. The immune system may trigger inflammation in other parts of the body when there is inflammation in the gut. These other problems tend to go when the gut symptoms settle, but not always.
How does Crohn's disease progress?
Crohn's disease is a chronic, relapsing condition. Chronic means that it is ongoing. Relapsing means that there are times when symptoms flare up (relapse), and times when there are few or no symptoms (remission). The severity of symptoms, and how frequently they occur, varies from person to person. The first episode (flare-up) of symptoms is often the worst.
What are the possible complications of Crohn's disease?
Complications may occur, particularly if flare-ups are frequent or severe. These include the following which often need treatment with surgery:
- Stricture. This is a narrowing of part of the gut. It is due to scar tissue that may form in the wall of an inflamed part of the gut. A stricture can cause difficulty in food passing through (a blockage). This leads to pain and vomiting.
- Perforation. This is a small hole that forms in the wall of the gut. The contents of the gut can then leak out and cause infection or an abscess inside the abdomen. This can be serious and life-threatening.
- Fistula. This is when the inflammation causes a channel to form between two parts of the body. For example, a fistula may form between a part of the small intestine and a part of the colon. Fistulas can also form between part of the gut and other organs such as the bladder or uterus (womb). The contents of the gut may then leak into these other organs. A perianal fistula sometimes develops. This is a fistula that goes from the anus or rectum and opens on to the skin near to the anus.
- Cancer. People with Crohn's disease have a small increased risk of developing cancer of the colon compared with the risk of the general population.
- Osteoporosis (thinning of the bones). The increased risk of this is related to the poor absorption of food that occurs in some people with severe Crohn's disease.
How is Crohn's disease diagnosed?
Depending on where the symptoms arise from, various tests may be done to confirm the diagnosis, and to determine how much of the gut is affected. For example, if you have symptoms coming from the colon or ileum, then a doctor may look inside the colon and ileum, using a special flexible telescope called a colonoscope. The colonoscope is passed through the anus, up into the colon, and a little further into the ileum. See separate leaflet called Colonoscopy for more detail.
The typical appearance of the inside lining of the colon or ileum suggests Crohn's disease. Biopsies (small samples) of the lining of various parts of the colon and ileum are usually taken. These are looked at under a microscope. The typical pattern of the cells may confirm the diagnosis.
If you have symptoms coming from the upper part of the gut, then a doctor may suggest a gastroscopy (endoscopy). This is where a thin, flexible telescope is passed down the oesophagus into the stomach. This allows a doctor or nurse to look inside. See separate leaflet called Gastroscopy (Endoscopy) for more detail.
A special X-ray of the large intestine (barium enema) or small intestine (barium meal) may be advised. Barium coats the lining of the gut and shows up as white on X-ray films. Typical patterns on the films show which parts of the gut are affected. Other tests such as an MRI or CT scan may be preferred, depending on which part of your bowel is affected, whether there are any complications and whether these tests are available in your area.
Also, blood tests are helpful from time to time to assess the level of inflammation within the gut, to check for anaemia and other deficiencies, and to assess your general well-being.
You may be asked to provide a stool sample for analysis to check for various germs that are sometimes present in people with Crohn's disease. Very occasionally, you may need to have an operation to diagnose Crohn's disease if your specialist cannot rule out an equally serious condition such as tuberculosis inside the abdomen.
What are the aims of treatment?
There are two main aspects of treatment:
- When a flare-up develops - a main aim is to clear symptoms. That is, to cause a remission of the disease.
- When a flare-up has settled - a main aim is to prevent any further flare-ups of symptoms. That is, to keep you in remission.
What are the treatment options for a flare-up of Crohn's disease?
The treatment advised can depend on various factors. For example, the severity of the symptoms, the site or sites of the inflammation in the gut, whether associated problems have developed, such as eye inflammation, and what treatments worked best for you in the past. Treatment decisions can become complex and a specialist will usually advise. Options that may be considered include the following:
This is an option for some people who have mild symptoms. There is a chance that the symptoms will settle on their own. If symptoms get worse, then decisions about treatment can be reviewed.
A course of steroids (corticosteroids)
Steroid medicines work by reducing inflammation. The two commonly used steroids for Crohn's disease are budesonide and prednisolone. In about 7 in 10 cases, symptoms are much improved within four weeks of starting steroids. The dose is reduced gradually, and then stopped once symptoms ease. A course of steroids for a few weeks is normally safe. Steroids are not usually continued once a flare-up has settled. The aim is to treat any flare-ups, but to keep the total amount of steroid treatment over the years as low as possible.
Although steroid tablets are commonly used, a steroid enema or suppository is also an option for a mild flare-up confined to the lower large intestine. Steroid injections directly into a vein may be required for a severe flare-up.
Newer powerful medicines that suppress the immune system have become available in recent years. These have made a big impact on the treatment of Crohn's disease in recent years. They tend to be divided into two groups:
Immunomodulators. These are medicines that modify and suppress the immune system. They include azathioprine, mercaptopurine and methotrexate. They tend to be used in more severe cases and in those where steroid treatment has not helped much.
Biological therapies. These are genetically engineered proteins such as special antibodies called monoclonal antibodies. These can target specific chemicals of the immune system, involved in the inflammation process. In Crohn's disease, a chemical called cytokine tumour necrosis factor alpha (TNF-alpha) is involved in the inflammation process. Medicines called infliximab and adalimumab (which are really manufactured antibodies) block the action of this chemical and therefore suppress the disease activity. Treatment with infliximab or adalimumab is an option in some cases - for example, in people who do not respond to steroid medication or to immunomodulators, or in certain situations causing severe symptoms. These medicines need to be given directly into a vein but then typically persist in the body for many weeks with long-lasting effects. People on these medicines should have their disease assessed every twelve months to see whether they still need them.
Aminosalicylate medicines are only sometimes used for Crohn's disease. (This is unlike in ulcerative colitis, a related condition, where they are used more commonly.) They include mesalazine, olsalazine, balsalazide and sulfasalazine. The exact way these medicines work is not clear but they are thought to counter the way inflammation develops in Crohn's disease. The active ingredient of each of these medicines is 5-aminosalicylic acid. However, each medicine is different in how the active ingredient is released or activated in the gut. Mesalazine is the most commonly used. Each of these medicines comes in different brand names and different preparations, such as oral tablets, sachets or suspension, liquid or foam enemas, or suppositories. The type of preparation (for example, tablets or enemas) may depend on the main site of the inflammation in the gut.
Antibiotics may need to be added to other treatments if infective complications are suspected - for example, if you develop an infected fistula such as an infected perianal fistula.
A very strict liquid diet that contains basic proteins and other nutrients has been found to help in some cases. This is called an elemental diet and is mainly used in children. A flare-up can settle within four weeks in some people who have this diet. After this, a normal diet is gradually restarted. It is not clear why this treatment works. It may have some effect of 'resting' the gut. This may be an alternative for some people when medication has not worked so well, or has caused bad side-effects. However, it is a controversial treatment.
An operation to remove a severely affected section of gut may be needed if other treatments do not work. The gut is cut above and below the affected part which is removed. The two ends are then joined up. Surgery is also usually needed to treat complications such as fistulas, strictures and abscesses.
- Iron tablets may be prescribed if you develop anaemia.
- Vitamins and other nutrient supplements may be needed if a large part of the gut is affected and food is poorly absorbed.
- Nutritional support such as dripping nutrients directly into a vein (parenteral nutrition) may be needed in severe cases.
- Painkillers may be needed for a while during flare-ups.
- Hospital admission for intravenous fluids (drip) and intensive treatment may be needed if you have a severe flare-up.
- Vaccinations may be offered to people with Crohn's disease, to protect them from a variety of infections, especially if they are on treatment which stops their immune system from working properly.
What are the treatment options to prevent flare-ups of symptoms?
Once a flare-up has settled, without treatment, on average there is about a 1 in 2 chance that another flare-up will develop within a year. Certain factors increase the likelihood of more severe and more frequent flare-ups.
For example, the severity of the first flare-up, the extent of the disease in your gut, your age, and the extent of treatment needed to control the initial flare-up. For some people it may not be worthwhile taking regular medication if flare-ups are not frequent, or are mild, and respond well to treatment when they occur. For others, medication to prevent flare-ups can make a big difference to quality of life.
The treatment options that may be considered to prevent flare-ups (which in medical language is to maintain remission) include the following:
- Regular mesalazine (mentioned earlier). This is less commonly used than previously as it is not considered effective in many cases. However, it still has a role in some cases, particularly to maintain remission after having a part of your gut removed as a treatment for a flare-up.
- A regular dose of an immunomodulator (described earlier). This is becoming more widely used as a treatment to prevent flare-ups.
- A regular dose of a biological therapy (described earlier). For example, an infusion of infliximab every eight weeks. This may be used in selected cases where flare-ups are severe and other treatments have not worked so well.
Each of the above treatments increases the chance of remaining free of flare-ups, but they do not always work. There is a balance between the likely benefits and the possible side-effects that occur in some people. Your doctor will advise about the pros and cons of long-term medication and which medication is best for your circumstances. Note: steroid medication is not generally used long-term to prevent flare-ups.
For smokers, giving up smoking may reduce the number and severity of flare-ups. It would always be wise to try to give up smoking. There are treatments that can help smokers to quit. Ask your doctor for advice on this.
The treatment of Crohn's disease is an evolving field. Various new medicines are under investigation and may change the treatment options over the next ten years or so.
Crohn's disease and pregnancy
If you have Crohn's disease and are planning to become pregnant, it is advised that you discuss this in advance with your doctor. For example, you may need extra folate supplements, and certain medicines which may be used for Crohn's disease, such as methotrexate, must not be used during pregnancy.
What is the outlook (prognosis)?
The outlook is variable. It depends on which part or parts of the gut are affected and how often and how severe the flare-ups are. Without treatment:
- About 3 in 20 people with Crohn's disease have frequent and/or severe flare-ups.
- A few people would have just one or two flare-ups in their lives, but for most of their lives have no symptoms.
- Most people would fall somewhere in between, have flare-ups from time to time, but can have long spells without symptoms.
Sometimes a severe flare-up is life-threatening and a small number of people die as a result of a serious complication such as a perforated gut.
Modern immunosuppressant medicines have made a big impact in recent years. Recent reports suggest that about 15 in 20 people with Crohn's disease remain in work ten years after diagnosis. So, this means that, in the majority of cases, with the help of treatment, the disease is manageable enough to maintain a near-normal life. However, the burden of the disease can be heavy for some people with severe disease.
Up to 8 in 10 people with Crohn's disease require surgery at some stage in their life for a complication. In about half of people with Crohn's disease, surgery is needed within the first ten years of developing the disease. The most common reason for surgery is to remove a stricture that has formed. Some people need several operations in their lifetime. If you develop Crohn's disease as a young adult, on average you can expect to have two to four operations in your lifetime. However, there is some evidence that the rate of surgery is coming down, probably due to the more modern treatments with medicines now available.
Crohn's disease and cancer of the colon
If you have Crohn's disease that affects at least half the surface of your large intestine (colon), you will be at a slightly increased risk of developing cancer.
People with this risk are usually advised to have their large intestine routinely checked after having had Crohn's disease for about ten years. This involves a look into the large intestine by a flexible telescope (colonoscopy) every now and then and taking small samples of bowel (biopsies) for examination. It is usually combined with chromoscopy (the use of dye spray which shows up suspicious changes more easily). Depending on the findings of this test and other factors such as the amount of intestine affected, whether you have had complications such as polyps and whether you have a family history of cancer, you will be put into a low, intermediate or high risk.
The National Institute for Health and Clinical Excellence (NICE) recommends the next colonoscopy/chromoscopy should depend on the degree of risk of developing colon or rectal cancer, as follows:
- Low - five years
- Intermediate - three years
- High - one year
After the next test, your risk will be calculated again.
What is inflammatory bowel disease?
When doctors talk of inflammatory bowel disease they usually mean people who either have Crohn's disease or ulcerative colitis. Both these conditions can cause inflammation of the colon and rectum (large bowel or large intestine) with similar symptoms, such as bloody diarrhoea, etc. Although these conditions are similar and treatments are similar, there are differences. For example, the inflammation of ulcerative colitis tends to be just in the inner lining of the gut, whereas the inflammation of Crohn's disease can spread through the whole wall of the gut. Also, ulcerative colitis only affects the colon and rectum, whereas Crohn's disease can affect any part of the gut. See separate leaflet called Ulcerative Colitis for more detail.
However, about 1 in 20 people with inflammatory bowel disease affecting just the colon cannot be classified as having either Crohn's disease or ulcerative colitis because they have some features of both conditions. This is sometimes called indeterminate colitis.
Note: inflammatory bowel disease is sometimes shortened to IBD. This is not the same as IBS which is short for irritable bowel syndrome - a very different disease.
Further help & information
Dr Tim Kenny
Dr Hayley Willacy
Dr John Cox