CHAPTER 1 Inflammatory bowel disease Inflammatory bowel disease

„ refers to two chronic diseases that cause inflammation of the intestines: ulcerative and Crohn's disease.

„ Although the diseases have some features in common, there are some important differences. Inflammatory bowel disease

„ Medical research hasn't determined yet what causes inflammatory bowel disease. But researchers believe that a number of factors may be involved, such as

„ environment

„ diet

„ possibly genetics Inflammatory bowel disease

„ Current evidence suggests that there's likely a genetic defect that affects how our immune system works and how the inflammation is turned on and off in those people with inflammatory bowel disease, in response to an offending agent, like: „ bacteria „ a virus „ or a protein in food Epidemiology of IBD

Ulcerative colitis Crohn’s disease Incidence (US) 11/100 000 7/100 000 Age of onset 15-30 & 60-80 15-30 & 60-80 Male:female ratio 1:1 1,1-1,8:1 Smoking May prevent May cause disease disease Oral contraceptive No increased risk Relative risk 1,9 Appendectomy Not protective Protective Monozygotic twins 8% concordance 67% concordance – microscopic features

„ Process is limited to the mucosa and submucosa with deeper layer unaffected

„ Two major histologic features: - the crypt architecture of the colon is distorted - some patients have basal plasma cells and multiple basal lymphoid aggregates Ulcerative colitis

„ is an inflammatory disease of the , also called the colon. In ulcerative colitis, the inner lining - or mucosa -of the intestine becomes inflamed and develops ulcers

„ is often the most severe in the rectal area, which can cause frequent . Ulcerative colitis – macroscopic features

„ 40-50% of patients have disease limited to the and rectosigmoid

„ 30-40% of patients have disease extending beyond the sigmoid

„ 20% of patients have a total colitis

„ Proximal spread occurs in continuity without areas of uninvolved mucosa Ulcerative colitis – macroscopic features

„ Mucosa is : - erythematous, has a granular surface that looks like a sand paper

„ In more severe diseases: - hemorrhagic, edematous and ulcerated

„ In fulminant disease a toxic colitis or a toxic may develop ( wall become very thin and mucosa is severly ulcerated) Ulcerative colitis – clinical presentation

„ The major symptoms of UC are: - diarrhea - rectal - tenesmus - passage of - crampy abdominal pain Ulcerative colitis – clinical presentation

„ Patients with usually pass fresh or blood- stained mucus either mixed with stool or streaked onto the surface of normal or hard stool

„ When the disease extends beyond the rectum, blood is usually mixed with stool or grossly bloody diarrhea may be noted

„ When the disease is severe, patients pass a liquid stool containing blood, pus, fecal matter

„ Other symptoms in moderate to severe disease include: anorexia, , vomitting, fever, weight loss Radiologic change of UC

„ Fine mucosal granularity

„ Mucosa become thickenned and superficial ulcers are seen (collar-button ulcers)

„ Loss of haustration Ulcerative colitis - complication

„ Hemorrhage

„ Perforation

„ Stricture

„ (transverse colon with a diameter of more than 5,0 cm to 6,0 cm with loss of haustration) UC – disease presentation

MILD MODERATE SEVERE

BOWEL < 4 per day 4-6 per day >6 per day MOVEMENTS

BLOOD IN small moderate Severe STOOL

FEVER none <37,5 C > 37,5 C

TACHYCARDIA none <90 mean >90 mean pulse pulse UC – disease presentation

MILD MODERATE SEVERE

ANEMIA mild >75% <75%

SEDIMENTATION <30mm >30mm RATE

ENDOSCOPIC Erythema, Marked erythema, Spontaneous APPEARANCE decreased vascular coarse granularity, bleeding, ulceration pattern, fine contact bleeding, no granularity ulceration Crohn’s disease

„ Crohn's disease differs from ulcerative colitis in the areas of the bowel it involves - it most commonly affects the last part of the and parts of the large intestine.

„ Crohn's disease isn't limited to these areas and can attack any part of the digestive tract

„ Crohn's disease generally tends to involve the entire bowel wall Crohn’s disease – macroscopic features

„ Can affect any part of GI tract from the mouth to the anus

„ 30-40% of patients have small bowel disease alone

„ 40-55% of patients have both small and large intestines disease

„ 15-25% of patients have colitis alone

„ In 75% of patients with small intestinal disease the terminal in involved in 90% Distribution of gastrointestinal Crohn's disease. Based on data from American Gastroenterological Association. Crohn’s disease – macroscopic features

„ CD is a transmural process

„ CD is segmental with skip areas in the midst of diseased intestine

„ In one –third of patients with CD perirectal , fissures, , anal stenosis are present Crohn’s disease – macroscopic features

„ mild disease is characterized by: aphtous or small superficial ulcerations

„ In more active disease: stellate ulcerations fuse longitudinally and transversely to demarcate island of mucosa that are histologically normal

„ Cobblestone appearance is characteristic of CD (both endoscopically and by barium radiography) Crohn’s disease – macroscopic features

„ Active CD is characterized by focal inflammation and formation of tracts

„ The bowel wall thickens and becomes narrowed and fibrotic, leading to chronic, recurrent Crohn’s disease – macroscopic features

„ Aphtoid ulceration and focal crypt abscesses with loose aggregation of macrophages which form granulomas

„ Transmural inflammation that is accompanied by fissures that penetrate deeply into the bowel wall Crohn’s disease – sign and symptoms

„ Ileocolitis - right lower quadrant pain and diarhhea - palpable mass, fever and leucocytosis - pain is colickly and relieved by defecation

„ Jejunoileitis - inflammatory disease is associated with loss of digestive and absorptive surface Crohn’s disease – sign and symptoms

„ Colitis and perianal disease - low grade fever, malaise, diarrhea, crampy abdominal pain, sometimes - pain is caused by passage of fecal material through narrowed and inflamed segments of large bowel

„ Gastroduodenal disease - nusea, vomiting, epigastric pain - second portion of is more commonly involved than the bulb IBD is associated with variety of extraintestinal menifestation. Almost one-third of the patients have at least one. Extraintestinal manifestation

Dermatologic 1. Erythema nodosum occurs in up to 15% of CD patients and 10% of UC patients The lesions of EN are hot, red, tender nodules measuring to 5cm in diameter and are found on the anterior surface of the legs, ankles, calves, thighs and arms

2. Pyoderma gangrenosum (PG) is seen in 1 to 12% of UC patients and is less common in CD colitis. PG may occur years before the onset of bowel symptoms. Lesions are common on the dorsal surface of the feet and legs but may occur on the arms, chest and even face. Extraintestinal manifestation

Rheumatologic

Peripherial arthritis developes in 15 to 20% of IBD patients, is more common in CD. It is asymmetric, polyarticular and migratory. Most often affects large joints of the upper and lower extremities

Ankylosing spondylosis (AS) occurs in 10% of IBD.

Sacroilitis is symetrical, occurs equally in UC and CD, often asymptomatic Extraintestinal manifestation

Ocular

The incidence of ocular complications in IBM patients is 1 to 10% The most common is conjunctivitis, anterior uveitis, episcleritis Symptoms include: ocular pain, photophobia, blurred vision, Extraintestinal manifestation

Urologic

The most frequent genitourinary complications are: calculi, ureteral obstruction, fistulas The highest frequency of nephrolithiasis (10-20%) occurs in patients with CD. Patients with IBD have an increased prevelance of osteoporosis secondary to vitamin D deficiency, calcium malabsorbtion, malnutrition, corticosteroid use

More common cardiopulmonary manifestations include endocarditis, myocarditis, pleuropericarditis and interstitial lung disease. Different clinical features

UC Crohn’s disease Blood in stool Yes Occasionally Mucus Yes Occasionally Systemic Occasionally Frequently symptoms Pain Occasionally Frequently Abdominal mass Rarely Yes Perineal disease No Frequently Different clinical features

UC Crohn’s disease Fistulas No Yes Small intestine No Frequently obstruction Colonic Rarely Frequently obstruction Response to No Yes Recurrence after No Yes surgery Different endoscopic features

UC Crohn’s disease

Rectal sparing Rarely Frequently

Continuous Yes Occasionally disease „cobblestoning” No Yes

Granuloma on No Occasionally biopsy