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Crohn Disease

para>CD can mimic UC with continuous bowel involvement; 10-15% of cases are difficult to differentiate; diagnosed as IBD unclassified (IBD-U).  

TREATMENT


  • Disease severity: Crohn Disease Activity Index (CDAI)
    • Asymptomatic: spontaneously, after medical/surgical intervention, or while on steroids (CDAI <150)
    • Mild to moderate CD: ambulatory patients able to tolerate PO intake without dehydration, obstruction, or >10% weight loss. No abdominal tenderness, toxicity, or mass (CDAI 150 to 220)
    • Moderate to severe CD: Patients who have failed initial treatment or who continue to have mild symptoms such as fever, weight loss, and abdominal pain (CDAI 220 to 450).
    • Severe: persistent symptoms despite outpatient therapy with glucocorticoids and/or biologics, or fulminant disease (peritonitis, cachexia, intestinal obstruction, abscess) (CDAI >450)
  • General strategies
    • Step-up approach: Begin treatment with milder therapy (5-ASA, antibiotics) followed by more aggressive agents (steroids, immunomodulators, anti-TNF agents), as needed.
    • Top-down approach: Early management with immunomodulators and/or anti-TNF agents before patients receive steroids, become steroid-dependent, or require surgery.

GENERAL MEASURES


Additional therapies depend on location of disease.  
  • Oral lesions: triamcinolone acetonide in benzocaine and carboxymethyl cellulose or topical sucralfate for aphthous ulcers, cheilitis, and/or granulomatous sialadenitis
  • Gastroduodenal CD: no clinical trials, although slow-release mesalamine may be beneficial, as it is partially released in proximal small bowel. Case reports note success of anti-TNF therapies. Symptomatic relief possible from proton pump inhibitors, H2-receptor blockers, and/or sucralfate.
  • Ileitis: supplementation of fat-soluble vitamins, iron, B12, folate, and calcium to prevent bone loss
  • Treatment toxicity: pancreatitis, bone marrow toxicity, lymphoma, nonmelanoma skin cancer, infections (TB, histoplasmosis, others), malignancy

MEDICATION


First Line
  • Asymptomatic patients: observation alone
  • Mild CD
    • 5-Aminosalicylates have minimal role in CD management. They can be used for colonic CD without deep ulcerations or penetration/fibrostenosing disease (2)[C].
    • Antibiotics use is controversial. Controlled trials have not consistently demonstrated efficacy (2)[C].
    • Glucocorticoid therapy: controlled ileal release budesonide (9 mg/day for 8 to 16 weeks, then discontinued over 2 to 4-week taper) for distal ileum and/or right colon involvement (2,3)[A]
    • Consider adjunctive therapy: antidiarrheals (loperamide); bile acid-binding resin (cholestyramine 4 to 12 g/day); probiotics (either alone or in combination may prevent recurrent inflammation and reduce symptoms in acute CD).
    • Induction/maintenance: 5-ASA is not recommended (3)[C]. Controlled ileal release budesonide, 9 mg/day, is effective for maintenance for up to 6 months (2)[A].
  • Moderate to severe CD
    • Induction: prednisone 40 to 60 mg/day (2)[A] or controlled-release budesonide (for isolated, moderate ileitis) or anti-TNF agents as initial induction agent or for lack of response to corticosteroid or immunomodulator (2,3)[A]
    • Maintenance: no role for mesalamine. If steroids required for induction, use immunomodulator (2,3)[B] or biologic (anti-TNF agent) (2)[A],(3)[B] for maintenance.
    • Except for budesonide, do not use steroids for maintenance (1)[A].
  • Severe disease: immunomodulators, anti-TNF agents ± steroids
    • Azathioprine or 6-mercaptopurine: thiopurine methyltransferase (TPMT) and LFTs prior to initiation. Check CBC/LFTs q2-3mo.
    • Methotrexate: effective for steroid-dependent and steroid-refractory CD (2)[B]
      • Folic acid 1 mg/day; follow LFTs
    • Anti-TNF therapies: active disease, fistulae, steroid sparing, some extraintestinal disease. Infliximab, adalimumab, certolizumab pegol
      • Check for evidence of TB and HBV infection prior to initiation of anti-TNF therapy.
      • Avoid live vaccines.
      • Monitoring: Consider anti-drug Ab levels to assess for immunogenicity. Serum concentrations of anti-TNF agents may also correlate with disease activity.
  • Combination therapy
    • Azathioprine + infliximab is more effective than either alone if no previously treatment with either.
    • Rare complication: hepatosplenic T-cell lymphoma (fatal, mostly seen in young males)
  • Anti-adhesion molecules: prevent inflammatory cells from entering GI tract
    • Vedolizumab: gut-specific, can be used in anti-TNF failures or anti-TNF naive patients as induction and maintenance; given IV, no risk of progressive multifocal leukoencephalopathy (PML); FDA approved May 2014
    • Natalizumab: non-gut-specific, PML risk (1/1,000). Can minimize risk by testing for John Cunningham (JC) virus antibody. However, can avoid risk of PML now with vedolizumab.

ADDITIONAL THERAPIES


Complications  
  • Peritonitis: bowel rest and antibiotic therapy (7 to 10 days parenteral antibiotics, followed by 2- to 4-week course of PO ciprofloxacin and metronidazole); surgery, as indicated
    • Consider holding steroids which mask sepsis.
  • Abscess: antibiotics, percutaneous drainage, or surgery with resection of affected segments
  • Small bowel obstruction: IV hydration, nasogastric (NG) suction, total parenteral nutrition (TPN) for malnutrition, resolution typically in 24 to 48 hours. Surgery for nonresponders

ONGOING CARE


FOLLOW-UP RECOMMENDATIONS


Patient Monitoring
Vaccinations in CD  
  • Check titers; avoid live vaccines (MMR, varicella, zoster) in patients on immunosuppressive therapy (steroids, 6MP, AZA, MTX, or anti-TNF).
  • Regardless of immunosuppression: HPV, influenza, pneumococcal, meningococcal, hepatitis A, B; Tdap

PATIENT EDUCATION


Crohn and Colitis Foundation of America (800) 343-3637; www.ccfa.org  

REFERENCES


11 Baumgart  DC, Sandborn  WJ. Crohn's disease. Lancet.  2012;380(9853):1590-1605.22 Lichtenstein  GR, Hanauer  SB, Sandborn  WJ, et al. Management of Crohn's disease in adults. Am J Gastroenterol.  2009;104(2):465-483.33 Talley  NJ, Abreu  MT, Achkar  JP, et al. An evidence-based systematic review on medical therapies for inflammatory bowel disease. Am J Gastroenterol.  2011;106(Suppl 1):S2-S25.

ADDITIONAL READING


Bernstein  CN. Treatment of IBD: where we are and where we are going. Am J Gastroenterol.  2015;110(1):114-126.  

CODES


ICD10


  • K50.919 Crohn's disease, unspecified, with unspecified complications
  • K50.00 Crohn's disease of small intestine without complications
  • K50.10 Crohn's disease of large intestine without complications
  • K50.019 Crohn's disease of small intestine with unsp complications
  • K50.812 Crohn's disease of both small and lg int w intestinal obst
  • K50.114 Crohn's disease of large intestine with abscess
  • K50.80 Crohn's disease of both small and lg int w/o complications
  • K50.913 Crohn's disease, unspecified, with fistula
  • K50.111 Crohn's disease of large intestine with rectal bleeding
  • K50.819 Crohn's disease of both small and lg int w unsp comp
  • K50.013 Crohn's disease of small intestine with fistula
  • K50.112 Crohn's disease of large intestine w intestinal obstruction
  • K50.912 Crohn's disease, unspecified, with intestinal obstruction
  • K50.911 Crohn's disease, unspecified, with rectal bleeding
  • K50.918 Crohn's disease, unspecified, with other complication
  • K50.811 Crohn's disease of both small and lg int w rectal bleeding
  • K50.814 Crohn's disease of both small and large intestine w abscess
  • K50.813 Crohn's disease of both small and large intestine w fistula
  • K50.113 Crohn's disease of large intestine with fistula
  • K50.118 Crohn's disease of large intestine with other complication
  • K50.011 Crohn's disease of small intestine with rectal bleeding
  • K50.014 Crohn's disease of small intestine with abscess
  • K50.119 Crohn's disease of large intestine with unsp complications
  • K50.914 Crohn's disease, unspecified, with abscess
  • K50.018 Crohn's disease of small intestine with other complication
  • K50.012 Crohn's disease of small intestine w intestinal obstruction
  • K50.818 Crohn's disease of both small and lg int w oth complication

ICD9


  • 555.9 Regional enteritis of unspecified site
  • 555.0 Regional enteritis of small intestine
  • 555.1 Regional enteritis of large intestine
  • 555.2 Regional enteritis of small intestine with large intestine

SNOMED


  • 34000006 Crohn's disease (disorder)
  • 397173003 Crohn's disease of intestine
  • 7620006 Crohn's disease of large bowel
  • 56689002 Crohn's disease of small intestine
  • 196977009 Crohn's disease of terminal ileum (disorder)
  • 71833008 Crohn's disease of small AND large intestines

CLINICAL PEARLS


  • The incidence of CD has risen over the past 4 decades.
  • MRE allows assessment of luminal and extraluminal CD without radiation exposure.
  • Assess for TB and HBV infection prior to initiating anti-TNF therapy.
  • Cigarette smoking doubles the risk of developing CD; tobacco cessation may reduce frequency of flares and need for surgery.
  • Test for C. difficile infection when evaluating diarrhea in all CD patients.
  • Hospitalized CD patients require deep vein thrombosis prophylaxis.
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