Crohn's Disease

BASICS

DESCRIPTION
An idiopathic inflammatory disease of the small intestine (60%), the colon (20%) or both; involving all of the layers of the bowel, but most commonly involving the terminal ileum. It is a slowly progressive and recurrent disease with prominent involvement of multiple regions of the intestine with normal sections in between.
  • System(s) affected: Gastrointestinal
  • Genetics: 15% of patients have first-degree relatives with inflammatory bowel disease. Family members develop the disease with similar patterns and similar age of onset.
  • Incidence/Prevalence in USA:
    • More common in Caucasians than African-Americans or Asians
    • More common in Jews
    • 20-100/100,000 prevalence
  • Predominant age:
    • Most cases 15-25 age of onset
    • Second smaller peak in ages 55-65
  • Predominant sex: Female > Male (slightly)
SIGNS AND SYMPTOMS
  • All forms of Crohn's
    • Diarrhea occurs in most patients
    • Abdominal pain in two-thirds
    • Weight loss
    • Abdominal tenderness often less than expected, in view of symptoms
    • Abdominal mass (occasionally)
    • Fistula - perirectal, bladder, skin, vagina
    • Extraluminal disease (10%) skin, iritis, arthritis, sclerosing cholangitis
  • Small bowel disease only
    • Diarrhea prominent, including nocturnal
    • Vague abdominal pain frequent. Only half of patients with abdominal pain have associated tenderness, not relieved with evacuation and often aggravated by food.
    • Intestinal obstruction in 1/3. Cramping abdominal pain precedes for months.
    • Bleeding in 20%, rarely massive
    • Perianal disease, including fistulae
    • Internal fistulae
    • Arthritis 5%
  • Colon disease only
    • Diarrhea prominent, including nocturnal
    • Hematochezia
    • Abdominal pain in 1/2, often relieved by stooling
    • Perianal disease in 40%, fistulae
    • Weight loss prominent
    • Megacolon occurs in about 10%
    • Arthritis in 20%
    • Intestinal obstruction occasional
  • Colon and small bowel disease
    • Intestinal obstruction much more common
    • Arthritis 5%
CAUSES
  • Idiopathic
  • Aggravated by bacterial infection
  • Aggravated by inflammatory cascade
  • Aggravated by smoking cessation
RISK FACTORS
  • More cigarette smokers than expected

DIAGNOSIS

LABORATORY
  • Elevated sedimentation rate
  • Anemia common
  • Albumin decreased in severe cases
  • Serum electrolytes imbalance
  • Specific nutrient deficiency: B12, fat soluble vitamins, folate

Drugs that may alter lab results: Sulfa drugs may lower folate after years of administration
Disorders that may alter lab results: All tests are non-specific, similar degrees of disease from other causes produce similar changes

PATHOLOGICAL FINDINGS
  • Involvement of all layers of gut wall with inflammation in > 95% cases at least focal areas
  • Skip areas in 80% (a normal segment between two involved segments)
  • Granuloma in 15%
  • Fat hypertrophy following mesenteric vessels in 50% of small bowel disease
SPECIAL TESTS
Colonoscopy is most helpful. The colon is not uniformly involved. The typical lesion is nodular with undermined pus filled mucosal ulcers. Strictures commonly present, occasionally preventing passage of the endoscope. The terminal ileum often has aphthous ulcers and may have nodularity. Small bowel proximal to an anastomosis is a very common site of recurrence.
IMAGING
  • Barium x-rays - enema and small bowel
    • Loss of smooth mucosa, undermined ulcers prominent
    • Narrowed lumen in most involved segments of small bowel
    • Fistulae from involved segment to other bowel loops, bladder, vagina or external
    • Skip areas, multiple lesions common
    • Failure to reflux into ileum on barium enema (not specific)
    • Ulcers undermining mucosa
    • Small bowel ulcerated wall
    • Narrowed lumen
    • Fistula to other parts of intestine
  • Plain x-rays
    • Intestinal obstruction
    • Toxic patient with colon disease, toxic megacolon
    • Evaluation of arthritis
    • Sacroiliitis
  • CT Scans
    • Define thickening of bowel wall if lumen not narrowed
    • Define abscess cavities and fistulae
    • Identify extensive perirectal disease
DIAGNOSTIC PROCEDURES
  • Ileoscopy and enteroscopy
  • The constellation of barium-identified distribution of lesions, endoscopic findings, and biopsies usually establish the diagnosis
  • Biopsies of mucosa in involved areas usually compatible with diagnosis, but not diagnostic. Helps rule out other causes.

TREATMENT

APPROPRIATE HEALTH CARE
  • Outpatient customary, inpatient for complications or special treatments
  • Progressive disease - average patient requires surgery each 4-7 years
GENERAL MEASURES
  • Attention to maintaining weight and nutrition
  • Monitor severe cases for fat malabsorption
  • Perirectal disease, sitz baths, soap and water after stooling, surgical drainage of perirectal abscesses, surgical treatment of recurrent fistulae if medical management fails
  • Extracolonic disease (uveitis, arthritis, dermatitis, sclerosing cholangitis) managed as other diseases in that special area
  • Folate supplements often needed
SURGICAL MEASURES
  • Indications for surgery:
    • Severe recurrent hemorrhage
    • Inability to thrive
    • Abscess
    • Total or recurrent intestinal obstruction
    • Toxic megacolon or extensive disease
    • Symptomatic fistulae other than rectal
    • Failure of ostomy to function after ³1 year
ACTIVITY

Full activity as tolerated

DIET
  • Usually no restrictions
  • If fat malabsorption, diminish fat in diet
  • If strictures or recurrent obstruction, avoid highly fibrous substances
  • If diarrhea prominent, increase dietary fiber (sometimes recommended), decrease fat
PATIENT EDUCATION

An important part of management. Crohn's and Colitis Foundation of America Inc, 11th floor, Park Ave South, NY 10016, Phone (800)343-3637. Joining local chapters recommended.

FOLLOW UP

PREVENTION/AVOIDANCE
  • Ongoing care with available physician
  • Consultant for review; long term advice
POSSIBLE COMPLICATIONS
  • Progression nearly certain - both expansion of old lesions and new lesions occur
  • Recurrence after operation nearly certain, usually occurs in gut segment most proximal to anastomoses
  • Fistulae occur about 15% of patients; perirectal, cutaneous, enterovaginal, enterovesicular are all seen
  • Extraluminal disease occurs in 10% with skin, uveal tract, joint, and biliary tract disease most common. All fairly specific in pattern; do not parallel activity of the luminal disease.
  • Extensive colon disease associated with increased risk of adenocarcinoma
  • Colon perforation and massive bleeding
  • Toxic megacolon
EXPECTED COURSE AND PROGNOSIS
  • Average patient has surgery each 7 years; >4 surgeries, expect short-bowel syndrome
  • Expect disease to recur
  • Majority will have normal life (work, children, full activities); overall life is shortened

MISCELLANEOUS

ASSOCIATED CONDITIONS
  • Viral gastroenteritis may be more devastating
  • Arthritis of two types - similar to rheumatoid and spondylitis
  • Variety of skin lesions, erythema nodosum, non-specific rashes, pyoderma gangrenosum
  • Uveal tract disease rare but related
  • Sclerosing cholangitis in about 10%, manifest from mild liver test abnormalities including pericholangitis on biopsy to full syndrome
  • Pigment gallstones are increased with ileal disease
AGE-RELATED FACTORS

Pediatric: Rare
Geriatric: N/A
Others: Occurs at any age

PREGNANCY
  • Reversible male sterility after long time on sulfasalazine - folate defers
  • No contraindications to pregnancy
OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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