Crohn Disease
Basics
- Chronic, progressive inflammatory GI tract disorder; most common site terminal ileum (80%)
- Hallmarks: transmural inflammation, fibrotic strictures, fistulas, fissures, abscesses, skip lesions, rectal sparing
- Diverse presentations: ileitis, ileocolitis, isolated colitis
Epidemiology
- Incidence: 3-20 per 100,000 person-years (North America), rising globally
- Bimodal age: 15-30 years (peak), smaller second peak 50-80 years
- Slight female predominance; increased incidence in northern climates
- Prevalence: 247 per 100,000 persons
Etiology and Pathophysiology
- Multifactorial: genetics, environment, microbial antigens, immune dysregulation causing inflammation and tissue injury
- 15% have first-degree relative with IBD (3- to 30-fold increased risk)
- Associated genetic syndromes: Turner, Hermansky-Pudlak, glycogen storage disease type 1b
Risk Factors
- Cigarette smoking doubles risk; cessation reduces flares and relapses
- Diet high in refined sugars, animal fat/protein, processed foods increases incidence
Commonly Associated Conditions
- Extraintestinal manifestations:
- Arthritis (20%), seronegative, may include ankylosing spondylitis, sacroiliitis (HLA-B27)
- Skin disorders (10%): erythema nodosum, pyoderma gangrenosum, psoriasis
- Ocular (5%): uveitis, iritis, episcleritis
- Kidney stones, osteopenia, osteoporosis, hypocalcemia
- Hypercoagulability: VTE prophylaxis essential during hospitalization
- Gallstones, primary sclerosing cholangitis (5%)
- Conditions linked to increased disease activity:
- Peripheral arthropathy, episcleritis, oral aphthous ulcers, erythema nodosum
- Complications:
- GI bleed, toxic megacolon, bowel obstruction/perforation, peritonitis, malignancy
- Intra-abdominal fistula, perianal disease
Diagnosis
History
- Hallmarks: crampy abdominal pain ± bleeding, prolonged diarrhea, fatigue, weight loss, fever, perianal disease
- Children: failure to thrive
- Exacerbating factors: infection, smoking, NSAIDs, antibiotics, stress
Physical Exam
- Signs of systemic inflammation/sepsis, malnutrition
- Abdominal tenderness, distension, rebound, guarding, palpable mass
- Perianal fistula, fissures, abscess
- Skin: erythema nodosum, pyoderma gangrenosum, psoriasis
Differential Diagnosis
- Acute abdomen: perforation, pancreatitis, appendicitis, diverticulitis, obstruction, stones, torsion
- Chronic diarrhea with pain: UC, radiation colitis, infections, ischemia, microscopic colitis, celiac, malignancy
- Wasting illness: malabsorption, malignancy
Diagnostic Tests and Interpretation
- Labs: CBC, chemistries, LFTs, ESR, CRP, iron, B12, vitamin D, stool calprotectin
- Stool studies: culture, C. difficile, ova/parasites if indicated
- Imaging: KUB for toxic megacolon
- Ileocolonoscopy with biopsy: highest sensitivity and specificity
- Upper endoscopy if upper GI symptoms
- Small bowel imaging: CT/MR enterography preferred over small bowel follow through; capsule endoscopy for small bowel visualization
- Perianal disease: EUS or MRI pelvis, exam under anesthesia
- Distinguishing CD vs UC:
- CD: skip lesions, transmural, rectal sparing, granulomas, fistulas, RLQ pain, uncommon rectal bleeding
- UC: continuous colonic involvement including rectum, LLQ pain, common rectal bleeding
Treatment
Disease Activity and Severity Assessment
- Use Harvey Bradshaw Index or Crohn Disease Activity Index (CDAI)
- Risk stratify for progressive disease: younger age, extensive disease, severe endoscopy, perianal disease, prior surgery, stricturing/penetrating behavior, EIMs
Mild to Moderate CD
- Observation if asymptomatic and mild endoscopic disease
- Steroids (budesonide, prednisone taper) for induction
- No role for mesalamine or antibiotics
- Maintenance: stop therapy or consider immunomodulator/biologic
Moderate to Severe CD
- Induction: biologics preferred; short course steroids may be used for symptom relief
- Maintenance: biologics ± immunomodulators
- Immunomodulators: azathioprine, 6-MP (maintenance only), methotrexate (steroid-dependent)
- Biologics:
- Anti-TNF agents: infliximab, adalimumab, certolizumab pegol (induction and maintenance)
- Gut-selective: vedolizumab
- Anti-IL-12/23: ustekinumab
- Anti-IL-23: risankizumab
- JAK inhibitor: upadacitinib (post anti-TNF failure; monitor shingles risk)
- Combination therapy reduces immunogenicity and improves efficacy
Additional Therapies
- Oral lesions: topical corticosteroids or sucralfate
- Gastroduodenal CD: PPIs, H2 blockers, sucralfate, anti-TNF therapy case reports
Surgery and Multidisciplinary Care
- Collaboration between surgery, gastroenterology, nutrition, radiology, pathology
Admission/Inpatient
- DVT prophylaxis essential
Ongoing Care
- Monitor CBC, BMP, LFTs every 3-4 months
- Monitor disease activity with CRP, calprotectin every 6 months
- TPMT/NUDT15 genotyping before thiopurines; therapeutic drug monitoring for infliximab
- Vaccinations: HPV, influenza, pneumococcal, meningococcal, hepatitis A/B, Tdap, varicella; avoid live vaccines on immunosuppressants
- Shingles vaccine recommended for immunosuppressed ≥18 years
- Colonoscopy with biopsies every 1-5 years after 8-10 years of colonic disease; annual in PSC patients
- Annual pap smears and skin exams for immunocompromised
- Bone health assessment and supplementation
Diet
- Mediterranean diet recommended
Patient Education
- CrohnsandColitisFoundation.org
- IBDandMe.org
Clinical Pearls
- Smoking doubles risk; cessation reduces flares and surgery
- MRE preferred for luminal and extraluminal assessment without radiation
- Test for TB and HBV before biologics
- Screen for C. difficile in diarrhea
- Hospitalized CD patients need DVT prophylaxis
- Anti-TNF delays/prevents postoperative recurrence
- Disease severity guides therapy more than activity
ICD10: - K50.0 Crohn’s disease of small intestine - K50.113 Crohn’s disease of large intestine with fistula - K50.011 Crohn’s disease of small intestine with rectal bleeding
Clinical Pearls: - Cigarette smoking doubles the risk of CD; cessation reduces flares and need for surgery. - MRE allows assessment of luminal and extraluminal disease without radiation exposure. - Assess TB and HBV prior to biologic therapy initiation. - Test for C. difficile infection when evaluating diarrhea in CD. - Hospitalized CD patients require DVT prophylaxis. - Anti-TNF therapy is effective in delaying or preventing postoperative recurrence. - Disease severity rather than disease activity should drive therapy decisions.