Inflammatory bowel disease (IBD), which includes Crohn disease and ulcerative colitis, is a relapsing and remitting condition characterized by chronic inflammation at various sites in the gastrointestinal tract, which results in diarrhea and abdominal pain.
Inflammation results from a cell-mediated immune response in the gastrointestinal mucosa. The precise etiology of inflammatory bowel disease is unknown, but evidence suggests that the normal intestinal flora inappropriately trigger an immune reaction in patients with a multifactorial genetic predisposition (perhaps involving abnormal epithelial barriers and mucosal immune defenses). No specific environmental, dietary, or infectious causes have been identified. The immune reaction involves the release of inflammatory mediators, including cytokines, interleukins, and tumor necrosis factor.
Although Crohn disease and ulcerative colitis are similar, they can be distinguished in most cases (see table Differentiating Crohn Disease and Ulcerative Colitis). About 10% of colitis cases are not initially distinguishable and are termed unclassified; if a surgical pathologic specimen cannot be classified, it is termed indeterminate colitis. The term colitis applies only to inflammatory disease of the colon (eg, ulcerative, granulomatous, ischemic, radiation-induced, infectious). Spastic (mucous) colitis is a misnomer sometimes applied to a functional disorder, irritable bowel syndrome.
Differentiating Crohn Disease and Ulcerative Colitis
Crohn Disease | Ulcerative Colitis |
|---|---|
Small bowel is involved in 80% of cases. | Disease is confined to the colon. |
Rectum is often spared; colonic involvement is usually right-sided. | Rectum is invariably involved; colonic involvement is usually left-sided. |
Gross rectal bleeding is rare, except in 75‒85% of cases of Crohn colitis. | Gross rectal bleeding is always present. |
Fistula, mass, and abscess development is common. | Fistulas do not occur. |
Perianal lesions are significant in 25‒35% of cases. | Significant perianal lesions never occur. |
On x-ray, bowel wall is affected asymmetrically and segmentally, with skip areas between diseased segments. | Bowel wall is affected symmetrically and uninterruptedly from rectum proximally. |
Endoscopic appearance is patchy, with discrete ulcerations separated by segments of normal-appearing mucosa. | Inflammation is uniform and diffuse. |
Microscopic inflammation and fissuring extend transmurally; lesions are often highly focal in distribution. | Inflammation is confined to mucosa except in severe cases. |
Epithelioid (sarcoid-like) granulomas are detected in bowel wall or lymph nodes in 25‒50% of cases (pathognomonic). | Typical epithelioid granulomas do not occur. |
Epidemiology
Inflammatory bowel disease (IBD) affects people of all ages but usually begins before age 30, with peak incidence from 14 to 24 (1). IBD may have a second smaller peak between ages 50 and 70; however, this later peak may include some cases of ischemic colitis.
IBD is most common among people of Northern European and Anglo-Saxon origin. It is 2 to 4 times more common among people of Ashkenazi Jewish ancestry (those from Central or Eastern Europe) than among non-Jewish White people from the same geographic location (2). The incidence is lower in central and southern Europe and lower still in South America, Asia, and Africa. However, the incidence is increasing among Black and Latin American people living in North America. Both sexes are equally affected. First-degree relatives of patients with IBD have a 4- to 20-fold increased risk; their absolute risk may be as high as 7%. Familial tendency is much higher in Crohn disease than in ulcerative colitis. Several gene mutations conferring a higher risk of Crohn disease (and some possibly related to ulcerative colitis) have been identified.
Cigarette smoking seems to contribute to development or exacerbation of Crohn disease but decreases risk of ulcerative colitis. Appendectomy done to treat appendicitis also appears to lower the risk of ulcerative colitis. Nonsteroidal anti-inflammatory drugs (NSAIDs) may exacerbate IBD. Oral contraceptives may increase the risk of Crohn disease. Some data suggest that perinatal illness and the use of antibiotics in childhood may be associated with an increased risk of IBD.
For unclear reasons, people who have a higher socioeconomic status may have an increased risk of Crohn disease.
General references
1. Bernstein CN, Rawsthorne P, Cheang M, et al: A population-based case control study of potential risk factors for IBD. Am J Gastroenterol 101(5):993-1002, 2006. doi: 10.1111/j.1572-0241.2006.00381.x
2. Schiff ER, Frampton M, Semplici F, et al: A new look at familial risk of inflammatory bowel disease in the Ashkenazi Jewish population. Dig Dis Sci 63(11):3049-3057, 2018. doi: 10.1007/s10620-018-5219-9
Extraintestinal Manifestations of Inflammatory Bowel Disease
Crohn disease and ulcerative colitis both affect organs other than the intestines. Most extraintestinal manifestations are more common in ulcerative colitis and Crohn colitis than in Crohn disease limited to the small bowel. Extraintestinal manifestations of inflammatory bowel disease are categorized in 3 ways:
1. Disorders that usually parallel (ie, wax and wane with) IBD flare-ups: These disorders include peripheral arthritis, episcleritis, aphthous stomatitis, and erythema nodosum. Arthritis tends to involve large joints and be migratory and transient.
2. Disorders that are clearly associated with IBD but appear independently of IBD activity: These disorders include ankylosing spondylitis, sacroiliitis, uveitis, pyoderma gangrenosum, and primary sclerosing cholangitis. Ankylosing spondylitis occurs more commonly in IBD patients with human leukocyte antigen B27 (HLA-B27). Most patients with spinal or sacroiliac involvement have evidence of uveitis and vice versa. Primary sclerosing cholangitis, which is a risk factor for cancer of the biliary tract, is strongly associated with ulcerative colitis and Crohn colitis. Cholangitis may appear before or concurrently with the bowel disease or even 20 years after colectomy. Liver disease (eg, steatotic liver disease, autoimmune hepatitis, pericholangitis, cirrhosis) occurs in 3 to 5% of patients, although minor abnormalities in liver tests are more common. Some of these conditions (eg, primary sclerosing cholangitis) may precede IBD by many years and, when diagnosed, should prompt an evaluation for IBD.
3. Disorders that are consequences of disrupted bowel physiology: These disorders occur mainly in severe Crohn disease of the small bowel. Malabsorption may result from extensive ileal resection and cause deficiencies of fat-soluble vitamins, vitamin B12, or minerals, resulting in anemia, hypocalcemia, hypomagnesemia, clotting disorders, and bone demineralization. In children, malabsorption retards growth and development. Other disorders include kidney stones resulting from excessive dietary oxalate absorption, hydroureter and hydronephrosis resulting from ureteral compression by the intestinal inflammatory process, gallstones resulting from impaired ileal reabsorption of bile salts, and amyloidosis secondary to long-standing inflammatory and suppurative disease.
Thromboembolic disease may occur as a result of multiple factors in all 3 categories.
Treatment of Inflammatory Bowel Disease
Supportive care
Biologic agents (anticytokine drugs)
Small-molecule agents (eg, Janus kinase inhibitor, sphingosine 1-phosphate [S1P] receptor modulator)
Sometimes antibioticsprobiotics
Several classes of medications are helpful for IBD. Details of their selection and use are discussed under each disorder and in Medications for Inflammatory Bowel Disease.
Supportive care
Most patients and their families are interested in diet and stress management. Although there are anecdotal reports of clinical improvement on certain diets, including one with rigid carbohydrate restrictions, controlled trials have shown no consistent benefit. Stress management may be helpful.
Health Maintenance in Inflammatory Bowel Disease
Immunizations
Patients with IBD should receive the yearly influenza vaccination and the pneumococcal vaccination. Those ≥ 19 years of age should receive the (1). This vaccine should be given before starting immunosuppressive therapy when possible.
Routine vaccinations such as tetanus-diphtheria, hepatitis A, hepatitis B, and human papillomavirus should be given as per the Advisory Committee on Immunization Practices (ACIP) guidelines.
The Centers for Disease Control and Prevention (CDC) and the Canadian Association of Gastroenterology recommend patients with IBD, including those on immunosuppressive therapy, receive an mRNA COVID-19 vaccine.
Screening tests
Female patients who are not receiving immunosuppressive therapy should be screened for cervical cancer every 3 years. Patients who are receiving immunosuppressive therapy should be screened for cervical cancer yearly.
All patients who are taking or plan to take immunomodulating drugs or biologic agents should be evaluated for skin cancer annually.
Patients at risk of osteoporosis should have a dual-energy x-ray absorptiometry (DXA) scan.
Health maintenance reference
1. Neil Murthy, A. Patricia Wodi, Sybil Cineas, et al: Recommended adult immunization schedule, United States, 2023. Ann Intern Med 176:367-380, 2023. doi:10.7326/M23-0041
More Information
The following English-language resources may be useful. Please note that THE MANUAL is not responsible for the content of these resources.
Advisory Committee on Immunization Practices (ACIP): Information about routine vaccination recommendations and guidelines and vaccine-specific updates
Canadian Association of Gastroenterology: Clinical practice guideline for immunizations in patients with inflammatory bowel disease—Part 2: Inactivated vaccines (2021)
