Inflammatory Bowel Disease, Emergency Medicine

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Inflammatory Bowel Disease, Emergency Medicine

Basics

Description

Vital sign stabilization as per BLS

If nonaccidental trauma is suspected, prompt referral to appropriate child protective agencies is required along with medical treatment.

Gastroenterologist or primary care as managing physician with surgical consultation as indicated

• Idiopathic, chronic inflammatory diseases of intestines, which can involve extraintestinal sites as well.
• Differentiation between ulcerative colitis (UC) and Crohns is not always clear; intermediate forms of inflammatory bowel disease (IBD) exist.
• May present as initial onset of disease or exacerbation of existing disease.
• Maintain high index of suspicion owing to frequent, subtle presentation of Crohn's disease.
• Pediatric considerations:Can occur in 1st few years of life.Extraintestinal manifestations may predominate.
• Differences between Crohn's and UC:Rectum almost always involved in UC with continuous inflammation proximally.Small intestine is not involved in UC.Crohn's can occur anywhere from mouth to anus, often with normal GI tract segments between affected areas.Crohn's involves transmural inflammation, whereas UC is confined to submucosa.
• Similarities between Crohn's and UC:Higher rate of colon cancer with disease >10 yr.Bimodal age distribution, with early peak between teens and early 30s and 2nd peak about age 60 yr.
• Crohn's disease clinical pattern:Ileocecal: ~40%Small bowel: ~30%Colon: ~25%Other: ~5%
• UC clinical pattern on presentation:Pancolitis: 30%:Most severe clinical course
• Proctitis or proctosigmoiditis: 30%:Relatively mild clinical course
• Left-sided colitis (up to splenic flexure): 40%:Moderate clinical course

• Can occur in 1st few years of life.
• Extraintestinal manifestations may predominate.

• Rectum almost always involved in UC with continuous inflammation proximally.
• Small intestine is not involved in UC.
• Crohn's can occur anywhere from mouth to anus, often with normal GI tract segments between affected areas.
• Crohn's involves transmural inflammation, whereas UC is confined to submucosa.

• Higher rate of colon cancer with disease >10 yr.
• Bimodal age distribution, with early peak between teens and early 30s and 2nd peak about age 60 yr.

• Ileocecal: ~40%
• Small bowel: ~30%
• Colon: ~25%
• Other: ~5%

• Pancolitis: 30%:Most severe clinical course

• Most severe clinical course

• Relatively mild clinical course

• Moderate clinical course

Etiology

• Unknown
• Crohns disease and UC are separate entities with common genetic predisposition.
• A positive family history is very common.
• Multifactorial origin involving interplay among the following factors:GeneticEnvironmentalImmune
• Pathogenesis:Gut wall becomes unable to downregulate its immune responses, ultimately resulting in chronic inflammation.
• There is no definitive evidence for the etiologic role of infectious agents.
• Psychogenic factors may play a role in some symptomatic exacerbations.

• Genetic
• Environmental
• Immune

• Gut wall becomes unable to downregulate its immune responses, ultimately resulting in chronic inflammation.

Diagnosis

Signs and Symptoms

• Crohns disease can present with any clinical correlates of chronic inflammatory, fibrostenotic, or fistualizing illness.
• UC may begin subtly or as catastrophic illness.
• Constitutional, GI, and extraintestinal manifestations are common with both Crohn's and UC.

• Constitutional:Crohns:Low-grade feverNight sweatsWeight lossFatiguePediatric: Growth or pubertal delayUC:Fever usually only in fulminant diseaseWeight loss/fatigue
• GI:Abdominal pain/tenderness-Crohn's disease:EpisodicPeriumbilical; may localize to right lower quadrant (RLQ) with ileal diseaseGeneralized with more diffuse intestinal involvementCan localize to area of intra-abdominal abscesses or fistulous involvementTenderness and distension suggest obstruction or toxic megacolonAbdominal pain/tenderness-UC:More generalized than Crohn's diseaseOften limited to predefecatory periodTenderness with distension-suspect toxic dilation
• Stool:Crohn's disease:Mild, loose stool, rarely >5/day~50% bloodyUC:Diarrhea is variable, can be severe.Vast majority are bloody, sometimes with severe hemorrhage.MucusTenesmus and urgency are common.
• Nausea/vomiting:Crohn's disease:Obstruction common with ileocolonic diseaseUC:Obstruction rareDiminished bowel sounds with toxic dilation
• Liver:Sclerosing cholangitis can be seen.Cholelithiasis can be seen in 35-60% of Crohn's.
• Renal:NephrolithiasisObstructive hydronephrosis
• Musculoskeletal:Peripheral arthritis/arthralgias-follows disease activity.Pediatric-may be confused with juvenile rheumatoid arthritis, idiopathic growth failure, anorexia nervosa.

• Crohns:Low-grade feverNight sweatsWeight lossFatiguePediatric: Growth or pubertal delay
• UC:Fever usually only in fulminant diseaseWeight loss/fatigue

• Low-grade fever
• Night sweats
• Weight loss
• Fatigue
• Pediatric: Growth or pubertal delay

• Fever usually only in fulminant disease
• Weight loss/fatigue

• Abdominal pain/tenderness-Crohn's disease:EpisodicPeriumbilical; may localize to right lower quadrant (RLQ) with ileal diseaseGeneralized with more diffuse intestinal involvementCan localize to area of intra-abdominal abscesses or fistulous involvementTenderness and distension suggest obstruction or toxic megacolon
• Abdominal pain/tenderness-UC:More generalized than Crohn's diseaseOften limited to predefecatory periodTenderness with distension-suspect toxic dilation

• Episodic
• Periumbilical; may localize to right lower quadrant (RLQ) with ileal disease
• Generalized with more diffuse intestinal involvement
• Can localize to area of intra-abdominal abscesses or fistulous involvement
• Tenderness and distension suggest obstruction or toxic megacolon

• More generalized than Crohn's disease
• Often limited to predefecatory period
• Tenderness with distension-suspect toxic dilation

• Crohn's disease:Mild, loose stool, rarely >5/day~50% bloody
• UC:Diarrhea is variable, can be severe.Vast majority are bloody, sometimes with severe hemorrhage.MucusTenesmus and urgency are common.

• Mild, loose stool, rarely >5/day
• ~50% bloody

• Diarrhea is variable, can be severe.
• Vast majority are bloody, sometimes with severe hemorrhage.
• Mucus
• Tenesmus and urgency are common.

• Crohn's disease:Obstruction common with ileocolonic disease
• UC:Obstruction rareDiminished bowel sounds with toxic dilation

• Obstruction common with ileocolonic disease

• Obstruction rare
• Diminished bowel sounds with toxic dilation

• Sclerosing cholangitis can be seen.
• Cholelithiasis can be seen in 35-60% of Crohn's.

• Nephrolithiasis
• Obstructive hydronephrosis

• Peripheral arthritis/arthralgias-follows disease activity.
• Pediatric-may be confused with juvenile rheumatoid arthritis, idiopathic growth failure, anorexia nervosa.

• Perianal:Crohns disease:Perianal abscessesFissures-characteristically painlessFistulas-seen in up to 50% of patients with colonic disease.May present prior to other manifestations.UC:No perianal involvement
• RLQ pain/mass often mistaken for appendicitis.
• Severe toxicity/abdominal pain-must exclude toxic megacolon.
• Extraintestinal:Eye:UveitisEpiscleritisKeratitisOral:Aphthous stomatitisDermatologic:Erythema nodosumPyoderma gangrenosum

• Crohns disease:Perianal abscessesFissures-characteristically painlessFistulas-seen in up to 50% of patients with colonic disease.May present prior to other manifestations.
• UC:No perianal involvement

• Perianal abscesses
• Fissures-characteristically painless
• Fistulas-seen in up to 50% of patients with colonic disease.
• May present prior to other manifestations.

• No perianal involvement

• Eye:UveitisEpiscleritisKeratitis
• Oral:Aphthous stomatitis
• Dermatologic:Erythema nodosumPyoderma gangrenosum

• Uveitis
• Episcleritis
• Keratitis

• Aphthous stomatitis

• Erythema nodosum
• Pyoderma gangrenosum

Essential Workup

• May present as initial onset of disease or exacerbation of existing disease.
• Maintain high index of suspicion because of subtle presentation of Crohns disease.

Diagnosis Tests & Interpretation

• Nothing diagnostic
• CBC:Anemia secondary to chronic or acute blood loss
• Electrolytes, BUN/creatinine, glucose
• Stool exam:Occult bloodClostridium difficileFecal leukocytes may be present.O & P and culture to rule out infectious cause of enteritis
• ESR is always elevated.
• Newer, investigational, serologic tests may have use as adjunctive diagnostic aids, screening tests, or predictors in therapy.

• Anemia secondary to chronic or acute blood loss

• Occult blood
• Clostridium difficile
• Fecal leukocytes may be present.
• O & P and culture to rule out infectious cause of enteritis

• Lifetime radiation dose is cumulative and IBD patients have repeated exposure; consider MRI when available.
• Upright chest and abdominal radiographs for:Toxic megacolon (>6 cm dilation)ObstructionAir in wall of colon (may indicate impending perforation)Perforation-subdiaphragmatic air or free air outlining liver or gall bladder
• CT abdomen/MRI:Distinguish abscess from localized inflammatory mass in Crohns.
• Colonoscopy with biopsy can confirm diagnosis of UC or Crohn's:Can be withheld with severe symptoms owing to perforation risk.
• Contrast imaging of small bowel, especially terminal ileum, may confirm diagnosis of Crohn's.
• MRI can be useful in Crohn's perianal disease and avoids ionizing radiation.

• Toxic megacolon (>6 cm dilation)
• Obstruction
• Air in wall of colon (may indicate impending perforation)
• Perforation-subdiaphragmatic air or free air outlining liver or gall bladder

• Distinguish abscess from localized inflammatory mass in Crohns.

• Can be withheld with severe symptoms owing to perforation risk.

Differential Diagnosis

• Infectious enteritis
• Pseudomembranous colitis (C. difficile)
• Appendicitis
• Diverticulitis
• Diverticulosis
• Functional bowel disease
• Lymphoma involving bowel
• Ischemic colitis
• Gonococcal or chlamydial proctitis
• HIV
• Colon cancer
• Vasculitis
• Amyloidosis

Treatment

Pre-Hospital

Initial Stabilization/Therapy

• IV 0.9% NS volume replacement if dehydrated
• Transfusion if significant blood loss

Ed Treatment/Procedures

• Nasogastric (NG) suction if obstruction or toxic dilation suspected
• Broad-spectrum antibiotics for fulminant UC or suspected perforation
• Consider steroid replacement if stress doses are required for those recently on oral steroids.
• Surgical evaluation indications:Free perforationIntestinal obstructionMassive, unresponsive hemorrhageToxic dilation:Not an absolute indication for surgeryIntensive medical management with small bowel decompression and close radiographic monitoring and surgical consultation
• Walled-off perforation with abscess:Usually not an indication for emergent surgeryCareful observation for peritonitis
• Medical therapy:Treatment is usually not initiated unless diagnosis is already established.Refill or restart medications in patient with known disease.ED-prescribed medical regimen should be individualized, and consultation with gastroenterologist strongly recommended:Aminosalicylate (sulfasalazine/mesalamine) in mild to moderate case.Antidiarrheal agent (diphenoxylate) is used-but withhold if severe disease or suspect toxic megacolon.Steroid (prednisone, budesonide or hydrocortisone enema, ACTH) is used for moderate to severe disease.Antibiotics (metronidazole and/or ciprofloxacin) aid in treatment of Crohns with colon/perineal involvement.Immunosuppressive agents (azathioprine, methotrexate) are used in severe disease.Monoclonal antibodies neutralize cytokine tumor necrosis factor (TNF)-α and inhibit binding to TNF-α receptors (infliximab [Remicade]). Used as parenteral therapy in disease unresponsive to other modalities. Not an ED drug, but be aware of potential severe adverse reactions, infusion reactions, autoimmune diseases, and infections.

• Free perforation
• Intestinal obstruction
• Massive, unresponsive hemorrhage
• Toxic dilation:Not an absolute indication for surgeryIntensive medical management with small bowel decompression and close radiographic monitoring and surgical consultation

• Not an absolute indication for surgery
• Intensive medical management with small bowel decompression and close radiographic monitoring and surgical consultation

• Usually not an indication for emergent surgery
• Careful observation for peritonitis

• Treatment is usually not initiated unless diagnosis is already established.
• Refill or restart medications in patient with known disease.
• ED-prescribed medical regimen should be individualized, and consultation with gastroenterologist strongly recommended:Aminosalicylate (sulfasalazine/mesalamine) in mild to moderate case.Antidiarrheal agent (diphenoxylate) is used-but withhold if severe disease or suspect toxic megacolon.Steroid (prednisone, budesonide or hydrocortisone enema, ACTH) is used for moderate to severe disease.Antibiotics (metronidazole and/or ciprofloxacin) aid in treatment of Crohns with colon/perineal involvement.Immunosuppressive agents (azathioprine, methotrexate) are used in severe disease.Monoclonal antibodies neutralize cytokine tumor necrosis factor (TNF)-α and inhibit binding to TNF-α receptors (infliximab [Remicade]). Used as parenteral therapy in disease unresponsive to other modalities. Not an ED drug, but be aware of potential severe adverse reactions, infusion reactions, autoimmune diseases, and infections.

• Aminosalicylate (sulfasalazine/mesalamine) in mild to moderate case.
• Antidiarrheal agent (diphenoxylate) is used-but withhold if severe disease or suspect toxic megacolon.
• Steroid (prednisone, budesonide or hydrocortisone enema, ACTH) is used for moderate to severe disease.
• Antibiotics (metronidazole and/or ciprofloxacin) aid in treatment of Crohns with colon/perineal involvement.
• Immunosuppressive agents (azathioprine, methotrexate) are used in severe disease.
• Monoclonal antibodies neutralize cytokine tumor necrosis factor (TNF)-α and inhibit binding to TNF-α receptors (infliximab [Remicade]). Used as parenteral therapy in disease unresponsive to other modalities. Not an ED drug, but be aware of potential severe adverse reactions, infusion reactions, autoimmune diseases, and infections.

Medication

• Ciprofloxacin: 500 mg (peds: 10-20 mg/kg q12) PO q12h
• Hydrocortisone enema: 60 mg per rectum
• Mesalamine enemas: 1-4 g retention enema-retain overnight. Adult.
• Mesalamine suppository: 500 mg per rectum BID. Adult.
• Mesalamine tablets:Asacol 800 mg PO TIDPentasa 1,000 mg PO QID
• Methylprednisolone: 125-250 mg IV load (peds: 2 mg/kg IV load, maintenance as adult), then 0.5-1 mg/kg/dose q6h for 5 days
• Metronidazole: 250-500 mg (peds: 30 mg/kg/24h) PO TID
• Prednisone: 40-60 mg (peds: 1-2 mg/kg) PO daily
• Sulfasalazine (Azulfidine): 500 mg (peds: 30 mg/kg) PO QID

• Asacol 800 mg PO TID
• Pentasa 1,000 mg PO QID

Follow-Up

Disposition

• Surgical indication:Massive, unresponsive hemorrhagePerforationToxic dilationObstruction
• Severe flare-up:Electrolyte imbalanceSevere dehydrationSevere painHigh feverSignificant bleeding

• Massive, unresponsive hemorrhage
• Perforation
• Toxic dilation
• Obstruction

• Electrolyte imbalance
• Severe dehydration
• Severe pain
• High fever
• Significant bleeding

• Initial presentation of diarrhea, mild pain, without toxicity, with close follow-up
• Mild to moderate exacerbation of known disease without obstruction, severe bleeding, severe pain, dehydration, with close follow-up, on renewed therapy or with addition of steroid

• Ocular
• Dermatologic

Followup Recommendations

Pearls and Pitfalls

• With severe flare, rule out toxic megacolon.
• Consider Crohns in children with growth/puberty delay.
• Consider Crohn's with perianal disease.
• Rule out C. difficile with flares; the incidence of C. difficile complicating IBD is increasing.
• Avoid antidiarrheals/spasmodic in severe UC.

Additional Reading

• Ananthakrishnan AN, Issa M, Binion DG. Clostridium difficile and inflammatory bowel disease. Gastroenterol Clin North Am. 2009;38(4):711-728.
• Sandborn WJ. New concepts in anti-tumor necrosis factor therapy for inflammatory bowel disease. Rev Gastroenterol Disord. 2005;5(1):10-18.
• Sauer CG, Kugathasan S. Pediatric inflammatory bowel disease: Highlighting pediatric differences in IBD. Med Clin North Am. 2010;94(1):35-52.
• Zisman TL, Rubin DT. Novel diagnostic and prognostic modalities in inflammatory bowel disease. Med Clin North Am. 2010;94(1):155-178.

See Also (Topic, Algorithm, Electronic Media Element)

• Abdominal Pain
• Diarrhea

Codes

ICD9

• 555.9 Regional enteritis of unspecified site
• 556.9 Ulcerative colitis, unspecified
• 558.9 Other and unspecified noninfectious gastroenteritis and colitis
• 555.2 Regional enteritis of small intestine with large intestine
• 555.0 Regional enteritis of small intestine
• 555.1 Regional enteritis of large intestine
• 555 Regional enteritis
• 556.1 Ulcerative (chronic) ileocolitis
• 556.2 Ulcerative (chronic) proctitis
• 556.3 Ulcerative (chronic) proctosigmoiditis
• 556.5 Left-sided ulcerative (chronic) colitis
• 556.6 Universal ulcerative (chronic) colitis
• 556.8 Other ulcerative colitis

ICD10

• K50.90 Crohns disease, unspecified, without complications
• K51.90 Ulcerative colitis, unspecified, without complications
• K52.9 Noninfective gastroenteritis and colitis, unspecified
• K50.80 Crohn's disease of both small and lg int w/o complications
• K50.00 Crohn's disease of small intestine without complications
• K50.10 Crohn's disease of large intestine without complications
• K51.00 Ulcerative (chronic) pancolitis without complications
• K51.20 Ulcerative (chronic) proctitis without complications
• K51.30 Ulcerative (chronic) rectosigmoiditis without complications
• K51.50 Left sided colitis without complications
• K51.80 Other ulcerative colitis without complications

SNOMED

• 24526004 Inflammatory bowel disease (disorder)
• 34000006 Crohns disease (disorder)
• 64766004 Ulcerative colitis (disorder)
• 71833008 Crohn's disease of small AND large intestines
• 13470001 Chronic ulcerative ileocolitis (disorder)
• 444548001 Ulcerative pancolitis (disorder)
• 445243001 Left sided ulcerative colitis (disorder)
• 56689002 Crohn's disease of small intestine
• 7620006 Crohn's disease of large bowel