We are pleased to welcome Rapha Medical Centre to iHealth. Their practice is situated at 150 Smith Road (Smith Road Centre), George Town. Tel. 769 5676 email: [email protected]

Overview

Overview Crohn’s disease, otherwise known as Regional Enteritis, may affect any part of the gastrointestinal tract from mouth to anus.

As shown in Table 1, Crohn’s disease most often involves the small intestines (79%). However one cannot overlook the fact that Crohn’s disease may significantly involve the colon (69%), with the colon solely being involved 19% of the time.

Case History

A fifty-six year old Caucasian male, who was initially treated abroad with antibiotics for traveler’s diarrhea, presented to our establishment with bloody diarrhea, fever and crampy abdominal pains.  He had these complaints for three weeks. Through a series of diagnostic procedures, which included colonoscopy, biopsy and histology, a diagnosis of Crohn’s disease of the colon was reached. Interestingly, the colonoscopy revealed multiple polyps throughout the colon which were characteristically pseudopolyps, rather than adenomatous polyps, which may become cancerous. This highlights the fact that colonic polyps may be inflammatory and a presentation of Crohn’s disease.  Hence, if patients in their fifties presents with blood in their stool, a colonoscopy must be done. This can reveal Crohn’s disease with pseudopolyposis and rectal sparing as in our patient. Alternate diagnoses include ulcerative colitis, diverticulitis, colonic polyposis and colon cancer among others.

Causes

Crohn’s disease is thought to be an autoimmune disease. Normally the immune system helps to protect the body. However, in an autoimmune disease, the immune system cannot tell the difference between the normal tissues of the body and foreign substances. The immune system therefore destroys the normal body tissues as well as foreign substances. For example, in Crohn’s disease, the body’s immune system attacks the gastrointestinal tract, causing inflammation. Diarrhea can result from this inflammation as was the case for our patient. There are three categories of disease presentation in Crohn’s disease namely stricturing, penetrating, and inflammatory. Stricturing disease causes narrowing of the bowel that may lead to bowel obstruction or changes in the caliber of the feces. Penetrating disease creates abnormal passageways (fistulae) between the bowel and other structures, such as the skin, vagina and anus. Inflammatory disease (or non-stricturing, non-penetrating disease) causes inflammation without causing strictures or fistulae. In the case of our patient, Crohn’s disease was of the inflammatory type since there was no evidence of stricture formation or penetration on colonoscopy.

Incidence

In the United States, the incidence (number of new cases) and prevalence (number of people who have the disease) have increased steadily during the last 50 years. The incidence of Crohn’s disease in the USA is approximately 149:100,000 as cited by the Crohn’s and Colitis Foundation of America. The incidence is thought to be similar in Europe but lower in Asia and Africa. The disease tends to affect people in their teens and 20s, and people in their 50s through to their 70s, and ages in between due to not being diagnosed with Crohn’s and being diagnosed instead with irritable bowel syndrome (IBS). Crohn’s disease is more prevalent in whites than in African Americans and Asians. In the United States, Europe, and South Africa, Crohn’s disease is 2-4 times more common among Jewish people than among other ethnic groups. A study done by Gastroenterologist Michael Lee in the 1980’s, showed that Crohn’s disease was rare in nearby Jamaica, and over a period of 20 years at the University Hospital, Kingston, Jamaica, twenty patients with Crohn’s disease were reported, with 14 female and 6 males. The mean age at presentation was 37 years (range 8-67), and symptoms were present for a mean of 12 months (range 1 day-4 yrs).

Risk Factors

As well as being thought to be an autoimmune disease, many factors play a role in causing Crohn’s disease. There is evidence of a genetic link to Crohn’s disease, putting individuals with siblings afflicted with the disease at higher risk. Males and females are equally affected. No risk factors were identified in our patient showing that the disease may occur sporadically.

History

The disease was named after an American gastroenterologist from New York City’s Mount Sinai Hospital, named Burrill Bernard Crohn, who, in 1932, together with two colleagues, described a series of patients with inflammation of the terminal ileum, the area most commonly affected by the illness. For this reason, the disease has also been called regional ileitis or regional enteritis. The condition, however, had previously been independently described in medical literature by others. The most notable case was in 1904 by Polish surgeon Antoni Leśniowski for whom the condition is alternatively named Leśniowski-Crohn’s disease in Polish literature. Inflammatory bowel diseases were described by Giovanni Battista Morgagni (1682–1771) and by Scottish physician T. Kennedy Dalziel in 1913.

Signs and symptoms

The symptoms may be subtle and it may be years before the disease is diagnosed. Usually symptoms begin between ages 15 and 30 years of age, but they may occur at any age. Table 2 shows signs and symptoms.

Signs and Symptoms

• Crampy abdominal pains
• fever
• vomiting
• constipation
• diarrhea with or without blood
• fatigue
• weight loss.

Crampy abdominal pains may be the first signs. Weight loss is usually due to the fact that they usually feel better when they do not eat. Also, if there is extensive small bowel involvement there may be malabsorption of carbohydrates and lipids, which again can cause weight loss.  The amount of diarrhea depends on whether the disease is mainly in the colon or in the small intestines. Therefore fecal consistency may range from watery to solid. One may have as much as twenty bowel movements per day, with the necessity to be awakened in the nights to pass stool. Mr. X’s main complaint was watery diarrhea. People with Crohn’s disease experience chronic recurring periods of flare-ups and remission. Among children, growth failure is common. Many children are first diagnosed with Crohn’s disease based on inability to maintain growth.

Signs outside of the Intestines

Extra-intestinal signs may be uveitis which is inflammation of the interior portion of the eye. This can cause pain especially when exposed to light. The inflammation may also involve the white portion of the eye (sclera) called episcleritis. Both can cause loss of vision if they are not treated. Crohn’s disease is also associated with joint pains which may include the knees, shoulders, sacroiliac joints and spines, as well as the small joints of the hands and feet. One form of skin involvement is called Erythema Nodosum. It presents as red nodules usually appearing on the shins.  Another skin manifestation is Pyoderma Gangrenosum which is a painful ulcerating nodule.

Crohn’s disease can also cause anaemia and osteoporosis. The mouth of the person with Crohn’s disease may also contain ulcers.

Diagnosis

Diagnosis of Crohn’s is sometimes challenging and a number of tests may be utilized to ascertain the diagnosis. Endoscopy in particular colonoscopy is the first diagnostic tool of choice. Colonoscopy is about 70% effective of diagnosing the disease while other modalities are less effective. Colonoscopy offers visualisation of the colon as well as the ileum or ending portion of the small intestines. Fig 3 shows the appearance of Crohn’s disease with pseudopolyposis on colonoscopy.

Colonoscopy done on Mr. X showed patchy lesions consistent with Crohn’s disease. About 21% – 60% Crohn’s disease shows granulomas on histology. However Mr. X’s biopsy of the colon did not reveal granulomas. Small bowel barium studies can be done to show areas affected in the small intestines. Small bowel studies was not performed on Mr. X. Additionally, the following results may also be altered in Crohn’s disease: Albumin, C-reactive protein, Erythrocyte sedimentation rate, fecal fat, hemoglobin, liver function tests and white blood cell count. Mr. X had increased ESR and CRP which are inflammatory markers. However he was not anemic and his Liver function tests and white blood cell count were within normal limits.

Complications

Complications include bowel obstruction, fistula formation, cholesterol gall stones, hypocalcaemia from fat malabsorption, perianal disease, and vitamin B12 malabsorption from terminal ileum involvement. Fortunately for Mr. X, none of theses complications had developed. This could be due to the fact that his condition was diagnosed before those complications could be developed.

Treatment

Though there is no known cure for Crohn’s disease, there are now suitable treatments for Crohn’s disease that are more likely to keep the disease in remission, so that if adequately controlled, Crohn’s disease may not significantly restrict daily living. Treatment for Crohn’s disease involves first treating the acute problem, then maintaining remission.

Medication

Acute treatment uses medications to treat any infection (normally antibiotics) and to reduce inflammation (normally aminosalicylate anti-inflammatory drugs and corticosteroids). When symptoms are in remission, treatment enters maintenance, with a goal of avoiding the recurrence of symptoms. Prolonged use of corticosteroids has significant side-effects; as a result, they are, in general, not used for long-term treatment. Alternatives include aminosalicylates alone, though only a minority are able to maintain the treatment, and many require immunosuppressive drugs. Medications used to treat the symptoms of Crohn’s disease include 5-aminosalicylic acid (5-ASA) formulations, prednisone, immunomodulators such as azathioprine, mercaptopurine, methotrexate, infliximab, adalimumab,  certolizumab and natalizumab. Hydrocortisone should be used in severe attacks of Crohn’s disease.

Surgery

Crohn’s cannot be cured by surgery, though it is used when partial or a full blockage of the intestine occurs. Surgery may also be required for complications such as obstructions, fistulas and/or abscesses, or if the disease does not respond to drugs. After the first surgery, Crohn’s usually shows up at the site of the resection, however it can appear in other locations. After a resection, scar tissue builds up, which can cause strictures, which form when the intestines become too small to allow stool to pass through easily, which can lead to a blockage. After the first resection, another resection may be necessary within five years. Short bowel syndrome (SBS, also short gut syndrome or simply short gut) can be caused by the surgical removal of the small intestines. It usually develops in those having had half or more of their small intestines removed. Diarrhea is the main symptom of short bowel syndrome.

Lifestyle Changes

Certain lifestyle changes can reduce symptoms, including dietary adjustments, proper hydration, and smoking cessation. Smoking may increase Crohn’s disease; stopping is recommended. Eating small meals frequently instead of big meals may also help with a low appetite. To manage symptoms, have a balanced diet with proper portion control. Fatigue can be helped with regular exercise, a healthy diet, and enough sleep. A food diary may help with identifying foods that trigger symptoms. Some patients should follow a low dietary fiber diet to control symptoms especially if fibrous foods cause symptoms. Patients should avoid milk or dairy products as they have been shown in recent research  to contribute to or even  cause Crohn’s disease.

Acutely Mr. X was treated antibiotics and intravenous steroids. He was discharged on Prednisolone, Azathioprine, Asocol (Mesalamine) and Predsol enema. He showed improvement.

Prognosis

Crohn’s disease is a chronic condition for which there is currently no cure. It is characterized by periods of improvement followed by episodes when symptoms flare up. With treatment, most people achieve a healthy weight, and the mortality rate for the disease is relatively low. However, Crohn’s disease is associated with an increased risk of small bowel and colorectal carcinoma, including bowel cancer. Our friend Mr. X has had a few flare ups which were able to be controlled. He presently lives like a regular person, and if you meet him on the road, you would not know that he has Crohn’s disease unless he tells you.