UC most commonly presents as bloody diarrhea with or without mucus. Patients commonly describe tenesmus, a sensation of incomplete evacuation, and abdominal pain. The physical exam may reveal predominantly left lower or left upper quadrant abdominal pain. Signs of an acute abdomen including guarding, rebound tenderness or percussion tenderness warrant investigation for toxic megacolon.

Presentations of Crohn’s disease vary considerably depending on the region of gastrointestinal involvement. Manifestations vary based on the underlying etiology of inflammation, fistula formation, or stricture formation. The symptom complex of right lower quadrant pain, weight loss, and non-bloody diarrhea are suggestive of Crohn disease flare-up. Fistula formation may result in fecaluria, pneumaturia, and rectovaginal fistulas.  Masses in the right lower quadrant suggest an abscess.

Affected children may present with growth retardation and/or delay in sexual maturation.

The World Gastroenterology Organization based symptoms for IBD:

• Diarrhea may be associated with blood or mucus; diarrhea may also occur at night and fecal incontinence is not uncommon
• Some patients with ulcerative colitis may present with constipation when the disease is localized to the rectum
• Abdominal pain, tenesmus, and severe urgency are also common presentations
• Crohn disease can present with RLQ pain and ulcerative colitis may present with LLQ pain
• Nausea and vomiting are more common in Crohn disease

Physical Exam

• Tachycardia, anxiety, fever, and dehydration are common
• Depending on the anemia, pallor may be noted
• Toxic megacolon may present with severe pain, fever, abdominal distension, chills, and lethargy. This surgical emergency should always be in consideration as it is fatal if missed.
• In Crohn disease, one may note anal fistulas, abscesses or even rectal prolapse.
• Occult blood on a digital rectal exam is common
• In children, one may only note growth retardation

Diagnosing IBD requires a combination of clinical findings, inflammatory laboratory markers, imaging findings, and endoscopic biopsies. Hematologic findings include microcytic anemia, leukocytosis, and thrombocytosis, Inflammatory markers such as the erythrocyte sedimentation rate (ESR) and high-sensitivity C-reactive protein (hsCRP) are commonly elevated.[7][8]

In some patients, the diagnosis may require ruling out parasitic disease like giardia, amebiasis, tuberculosis, and Strongyloides.

Complete blood count will identify anemia, leukocytosis and albumin levels.

Fecal calprotectin levels can be used as a marker for intestinal inflammation. Levels of perinuclear antineutrophilic cytoplasmic and anti-saccharomyces cerevisiae antibodies may be elevated in Crohn disease. Finally, stool studies must be done to rule out ova and parasitic organisms.

The abdominal x-ray can assess for the presence of free air, bowel obstruction or toxic megacolon.

Barium studies are done to characterize the bowel disease; a lead pipe appearance indicates ulcerative colitis; sparing of the rectum is indicative of Crohn disease and thumb printing is indicative of mucosal inflammation. Further, the barium studies may reveal skip lesions and stricture formation in the ileum, which are indicative of Crohn disease.

Ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI) have all been used in the diagnosis of IBD or to assess for complications. US usage in trained individuals can evaluate the right lower quadrant for ileal disease. MRI can evaluate for rectal fistulas. Most commonly, CT is employed to evaluate for perforation or bowel obstruction. CT enterography can be helpful in assessing for strictures or in operative planning.

Endoscopy evaluation with either esophagogastroduodenoscopy, colonoscopy, or both is essential to obtaining biopsies to confirm a diagnosis of IBD.

The goal of treatment is to induce remission for either UC or CD. Treatment of IBD is divided into the management of the mild, moderate, and severe disease. Agents formerly reserved for the more severe disease are now employed sooner. UC treatment depends greatly on the extent of disease and presence of extraintestinal manifestations. For those with mild to moderate disease limited to the rectum, aminosalicylate agents like mesalamine are mainstays. Mesalamine is administered rectally but may be combined with oral therapy to induce or maintain remission. For those patients with the moderate disease who are refractory to mescaline, oral glucocorticoids or immunomodulators such as TNF-a monoclonal antibodies (infliximab) may be an option. Up to 25% of all UC patients will require total colectomy for the uncontrolled disease. Proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the procedure of choice for elective cases.[9][10][11]

Flareups are usually managed with corticosteroid therapy. For those who have more than 1-2 flareups a year, the use of anti-TNF agents or other immunosuppressive is recommended.

CD treatment depends on the portion of the GI tract involved, the degree of fistulizing or structuring, and any extraintestinal complications. Treatment of mild ileocecal disease is usually begun with mesalamine, which can be further augmented with the use of oral budesonide, a steroid with significant first-pass metabolization to limit systemic side effects. For more extensive disease, systemic steroid therapy with prednisone is necessary. The goal is to wean these steroids within six weeks. In those patients who cannot wean, an immunomodulating agent is added.  6-mercaptopurine (Purinethol), azathioprine (Imuran), or low-dose methotrexate. In those patients with moderate to severe disease, anti-tumor necrosis factor (anti-TNF) should be initiated. Before initiating biologic therapy, patients must complete purified protein derivative (PPD) to assess for latent tuberculosis. Surgical treatment may be necessary for those with severe fistulizing disease including diverting ostomy.[1][12][13]

It is vital to assess the bone density in patients who are administered steroids; osteoporosis has significant morbidity in these patients. If steroid use for more than 3 months is expected, then calcium supplements and bisphosphonates should be introduced.

Stepwise Therapy

A stepwise approach that is used for IBD management is outlined below.

The first step in pharmacologic therapy for IBD is aminosalicylates. If the patient does not respond to an appropriate dose of aminosalicylates, the second step is the addition of corticosteroids, which tend to result in a significant decrease in inflammation. Once the response is seen, the dose can be tapered.

The immune-modifying agents (e.g Anti-TNF agents) are the step III drugs. These are used when the patient does not respond to corticosteroids, steroids are required for prolonged periods, or the steroids cannot be tapered down without recurrence of symptoms. 

Lately, a step-down approach is being favored more for patients with high-risk or severe disease. This includes the early introduction of higher step medications such as anti-TNF agents with rapid de-escalation when the response is seen. Step down approach improves patient outcomes and prevents complications in patients with high-risk or severe disease.Step IV includes clinical trial agents that tend to be disease-specific i.e some work only for ulcerative colitis and other for Crohn disease. Examples of these experimental agents include thalidomide and interleukin (IL)-11 for Crohn disease, butyrate enema, nicotine patch, and heparin for ulcerative colitis. Multiple contraindications and serious side effects are associated with these experimental agents.

In patients presenting with new diarrhea, infectious etiologies of diarrhea including parasites Escherichia coli 0157:H7 and Clostridium difficile must be ruled out first. Other colitis etiologies should be considered including, but not limited to, microscopic, lymphocytic, and collagenous. Those presenting with abdominal pain must have other causes considered as well including, but not limited to, appendicitis, irritable bowel disease, celiac disease and functional abdominal pain.

The objective measure that is most commonly used for Crohn disease is Crohn Disease Activity Index (CDAI). This index, which includes patients' demographic characteristics, subjective complaints and symptoms, laboratory values, and extraintestinal findings, classify the disease activity. Different online tools are available to calculate this score and help predict the disease stage. Another tool for Crohn disease is Harvey-Bradshaw Index (HBI). Similarly, such tools are also available for ulcerative colitis and include Simple clinical colitis activity index (SCCAI), Mayo Score / Disease Activity Index (DAI), and Seo index for disease quantification. 

The prognosis for both UC and CD depends on the extent of disease and treatment response. The stool markers lactoferrin and calprotectin are useful in determining postoperative recurrence of CD. Some evidence exists that these can also be used to predict future flares. However, patients with IBD tend to have much higher mortality compared to the general population. Causes of death include primary disease, infections, and respiratory illness. Heart disease is not a risk factor for death in IBD. Finally, psychological morbidity is very high in IBD patients and the quality of life is poor.

Continued surveillance for dysplasia is critical for long-standing UC patients. Cumulative risk of colorectal cancer is estimated to be as high as 30% for those with the disease of 30 years or more. The extraintestinal manifestation of primary sclerosing cholangitis leads to liver failure.[14][15]

 Intestinal

• Hemorrhage
• Strictures
• Colon perforation
• Anal fistulas
• Pelvic or perirectal abscesses
• toxic megacolon
• Cholangiocarcinoma, colon cancer

Extra Intestinal

• Osteoporosis
• Deep vein thrombosis
• Anemia
• Gallstones
• Primary sclerosing cholangitis
• Aphthous ulcers
• Arthritis
• Iritis
• Pyoderma gangrenosum

Patients with IBD need life long follow up for their disease. There is no particular diet or supplement that has been shown to delay or prevent the symptoms of the disease.

• UC and CD are differentiated histologically by the depth of involvement in the bowel wall.
• Both forms of IBD can develop with extraintestinal manifestations.

There are several gaps in our knowledge regarding the care of patients with IBD. Even though many advances have been made in treatment, a number of patients continue to miss out on these treatments. The best care for patients with IBD is from an interprofessional team that is dedicated to this pathology and is fully aware of the latest guidelines. Patients with IBD lead an unpredictable life with constant flare-ups and hence the interprofessional team should establish dedicated phone and support lines staffed by nurses and pharmacists. A network of healthcare providers should be easily accessible to deal with emergencies. Physician and allied healthcare collaboration are vital if one is to avoid delay in treatment. The goal is to halt the disease before widespread bowel damage and disability occurs. Furthermore, patient and healthcare communication should be improved and the patients should be supported during times of crises. Finally, evidence suggests that there is a great need to establish centers of excellence so that patients receive a consistent level of quality care across different healthcare settings.[16][17] (Level V)

Outcomes

The mortality of patients with IBD is about 1.5 -5 times higher compared to the general population. Patients with Crohn disease suffer the highest morbidity and mortality. The major cause of death include infections, the progression of disease, surgical complications, and multiorgan involvement. More important, patients with IBD also have a high rate of colorectal cancer. Patients who develop pancolitis have the highest risk of colon cancer within two decades. Hence screening colonoscopy is recommended at 1-2 year intervals.[18]

In addition to the disease, these patients are also managed with potent medications like steroids and biological agents, which have a host of adverse effects. Thus, the importance of the pharmacist who should be alert for any adverse reaction. Patients with IBD are also at risk for asthma or COPD.

Finally, IBD has enormous mental morbidity. Many patients develop depression, suicidal tendencies, and anxiety. thus, at every visit, the nurse should monitor the patient's mental status and make appropriate referrals.[19][20]

The quality of life for most patients is poor to okay.