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Crohn's Disease

Hendy Satrya Kurniawan

Definition
Crohn's disease is a chronic, idiopathic inflammatory disease with a propensity to affect the distal ileum, although any part of the alimentary tract can be involved United States : 3.6 to 8.8 per 100,000 there are 2-fold to 4- fold increased prevalence in Ashkenazi Jewish females > males. The median age at which patients are diagnosed approximately 30 years; however, age of diagnosis can range from early childhood through the entire life span.

Higher socioeconomic status increased risk Most studies have found breast-feeding to be protective against the development of Crohn's disease. Crohn's disease is more prevalent among smokers. Furthermore, smoking is associated with the increased risk for both the need for surgery and the risk of relapse after surgery for Crohn's disease.

Pathophysiology
Crohn's disease is characterized by sustained inflammation. Various hypotheses on the roles of environmental and genetic factors in the pathogenesis of Crohn's disease have been proposed. Many infectious agents have been suggested to be the causative organism of Crohn's disease.
Chlamydia, Listeria monocytogenes, Pseudomonas species, reovirus, Mycobacterium paratuberculosis, and many others.

Pathophysiology
a variety of defects in immune regulatory mechanisms, e.g., overresponsiveness of mucosal T cells to enteric flora-derived antigens, can lead to defective immune tolerance and sustained inflammation Specific genetic defects associated with Crohn's disease For example, the presence of a locus on chromosome 16 (the so-called IBD1 locus) has been linked to Crohn's disease. The IBD1 locus has been identified as the NOD2 gene.

Pathology
The earliest lesion characteristic is the aphthous ulcer.

These superficial ulcers are up to 3 mm in diameter and are surrounded by a halo of erythema. In the small intestine, aphthous ulcers typically arise over lymphoid aggregates.

Granulomas are highly characteristic of Crohn's disease up to 70% of intestinal specimens obtained during surgical resection

Pathology
As disease progresses, aphthae coalesce into larger, stellate-shaped ulcers, linear or serpiginous ulcers. With advanced disease, inflammation can be transmural. Serosal involvement results in adhesion of the inflamed bowel to other loops of bowel or other adjacent organs. Transmural inflammation also can result in fibrosis, with stricture formation, intra-abdominal abscesses, fistulas, and, rarely, free perforation. Inflammation in Crohn's disease can affect discontinuous portions of intestine: so-called "skip lesions" that are separated by intervening normal-appearing intestine.

fat wrapping

Crohn's disease vs ulcerative colitis : inflammation in ulcerative colitis is limited to the mucosa and submucosa but may involve the full thickness of the bowel wall in Crohn's disease inflammation is continuous and characteristically affects the rectum in ulcerative colitis but may be discontinuous and spare the rectum in Crohn's disease

Clinical Presentation
Signs and symptoms of Crohn's disease can range from mild to severe and may develop gradually or come on suddenly, without warning. They include:

Diarrhea Abdominal pain and cramping Blood in your stool UlcersReduced appetite and weight loss Fistula or abscess

The distal ileum is the single most frequently affected site, being diseased at some time in 75% of patients with Crohn's disease. The small bowel alone is affected in 15 to 30% of patients, Both the ileum and colon are affected in 40 to 60% of patients Colon alone is affected in 25 to 30% of patients. Isolated perineal and anorectal disease occurs in 5 to 10% of affected patients. Uncommon sites of involvement include the esophagus, stomach, and duodenum.

Clinical Presentation
25% of all patients with Crohn's disease will have an extraintestinal manifestation

Erythema nodosum, Pyoderma gangrenosum Peripheral arthritis, Ankylosing spondylitis, Sacroiliitis Conjunctivitis, Uveitis/iritis Hepatic steatosis, Cholelithiasis Primary sclerosing cholangitis, Pericholangitis Nephrolithiasis Thromboembolic disease, Osteoporosis Endocarditis, myocarditis, pleuropericarditis Interstitial lung disease Amyloidosis, Pancreatitis

Pyoderma gangrenosum

Diagnosis
acute or chronic abdominal pain, especially the right lower quadrant, chronic diarrhea, evidence of intestinal inflammation on radiography or endoscopy, the discovery of a bowel stricture or fistula arising from the bowel, and evidence of inflammation or granulomas on intestinal histology. radiographic, endoscopic, and pathologic tests

CROHNS DISEASE
Contrast radiograph demonstrating that the lumen of the terminal ileum is narrowed and markedly separated from the surrounding small bowel by a thickened wall. There are skip lesions in the colon that have a cobblestone appearance.

Management
Because no curative therapies are available for Crohn's disease, the goal of treatment is to palliate symptoms rather than to achieve cure. Medical therapy is used to induce and maintain disease remission. Surgery is reserved for specific indications In addition, nutritional support in the form of aggressive enteral regimens or, if necessary, parenteral nutrition, is used to manage the malnutrition that is common in patient's with Crohn's disease.

Medical Therapy
Pharmacologic agents used to treat Crohn's disease :
Antibiotics Aminosalicylates (sulfasalazine) Corticosteroids Immunomodulators

Surgical Therapy
Seventy to 80% of patients with Crohn's disease will ultimately require surgical therapy for their disease Surgery is generally reserved for patients whose disease is unresponsive to aggressive medical therapy or who develop complications One-third of patients with Crohn's disease will require surgery for intestinal obstruction. Abscesses and fistulas are frequently encountered during operations performed for intestinal obstruction in these patients,

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