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Inflammatory Bowel Disease

Chapter 337 | Part 10: Disorders of the Gastrointestinal System

KEY CLINICAL POINTS

  • IBD includes ulcerative colitis (UC) and Crohn’s disease (CD), with global incidence rising in urbanized regions due to environmental factors.
  • Genetic factors (e.g., NOD2, ATG16L1) and environmental triggers (diet, microbiome, smoking) interact to drive pathogenesis.
  • Differential diagnosis includes infectious causes (e.g., C. difficile, TB) and non-IBD conditions like ischemic colitis or diverticulitis.
  • Biologic therapies (anti-TNF, IL-12/23 inhibitors) are first-line for moderate-to-severe IBD, with surgical options for complications like strictures or fistulas.
  • Pregnancy management requires careful medication selection (e.g., avoiding methotrexate) and endoscopic surveillance for dysplasia/cancer.

1. DEFINITION & OVERVIEW

Inflammatory bowel disease (IBD) encompasses ulcerative colitis (UC) and Crohn’s disease (CD), characterized by chronic inflammation of the gastrointestinal tract. IBD is a multifactorial condition involving genetic susceptibility, environmental triggers, and immune dysregulation. The disease spectrum includes both localized and transmural inflammation, with distinct clinical and histopathologic features.

Table 337-1: IBD Epidemiology

UC CD
Age of onset: 2nd-4th decades; 7th-9th decades Age of onset: 2nd-4th decades; 7th-9th decades
Ethnicity: White > Black > Latinx > Asian Ethnicity: White > Black > Latinx > Asian
Smoking: protective for UC, risk factor for CD Smoking: risk factor for CD
Oral contraceptives: no increased risk for UC Oral contraceptives: hazard ratio 2.82 for CD
Appendectomy: protective for UC Appendectomy: modestly associated with CD
Monozygotic twins: 6-18% concordance for UC Monozygotic twins: 38-58% concordance for CD

1.1 Global Epidemiology

IBD prevalence is highest in North America (UC: 286/100,000; CD: 319/100,000), Europe (UC: 505/100,000; CD: 322/100,000), and Oceania. Incidence has increased in developing countries, with annual increases of 4-14% in Asia/Pacific regions. Urbanization and Western dietary patterns correlate with rising IBD rates.

Whites have highest prevalence; Latinx, Black, and Asian populations show increasing rates. Female-to-male ratios: UC (0.51-1.58), CD (0.34-1.65). Pediatric IBD (20-25% of cases) includes early-onset (VEO) and infantile IBD with genetic mutations (e.g., NOD2, ATG16L1).

2. EPIDEMIOLOGY

IBD affects 1-2% of the global population, with rising incidence in urbanized regions. The global prevalence is 0.3-0.5%, with higher rates in Western countries. Environmental factors (diet, hygiene, antibiotic use) and genetic predisposition interact to influence disease risk.

Table 337-2: Primary Genetic Disorders

Name Genetic Association Phenotype
Turner’s syndrome X chromosome deletion UC and colonic CD
Wiskott-Aldrich syndrome X-linked recessive Colitis, immunodeficiency, thrombocytopenia
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) Loss of FoxP3 transcription factor UC-like autoimmune enteropathy
Deficient IL-10/IL-10R IL-10 receptor dysfunction Severe, refractory IBD

Annual incidence rates: CD (4-14% increase), UC (4-10% increase). Asia-Pacific regions show highest incidence (1.5-3.6 cases/100,000). Urbanization and Western dietary patterns correlate with rising IBD rates.

2.2 Risk Factors

Smoking: protective for UC, risk factor for CD. Appendectomy: protective for UC. Oral contraceptives: increased risk for CD. Genetic factors (NOD2, ATG16L1, IL23R) contribute to susceptibility.

3. ETIOLOGY & PATHOPHYSIOLOGY

IBD results from complex interactions between genetic predisposition, environmental triggers, and immune dysregulation. The gut microbiome and host immune responses play critical roles in disease pathogenesis.

Table 337-3: Genetic Loci in IBD

Locus Associated Gene Function
IBD1 NOD2 Nucleotide-binding oligomerization domain 2
IBD2 ATG16L1 Autophagy-related protein 16-like 1
IBD3 IL23R Interleukin 23 receptor
IBD4 IRGM Immunity-related GTPase family M

3.1 Genetic Factors

Genetic loci include Norpion (NOD2), ATG16L1, IL23R, and IRGM. Mutations in these genes disrupt innate immunity and autophagy, increasing susceptibility to inflammation.

3.2 Microbiome and Immune Dysregulation

Dysbiosis and altered gut microbiota contribute to inflammation. The microbiome interacts with host genetics to influence disease severity. Immune responses involving Th1/Th17 cells and cytokines (TNF- α , IL-12/23) drive inflammation.

3.3 Environmental Triggers

Diet, smoking, antibiotic use, and gut permeability influence disease development. Western diets high in processed foods and low in fiber are associated with increased risk.

4. CLINICAL FEATURES

IBD presents with diverse symptoms depending on disease location and extent. UC typically involves continuous rectal involvement, while CD can affect any GI tract segment with transmural inflammation.

Table 337-4: Montreal Classification for UC

Location Behavior Coprology
Proctitis Continuous Mucous
Left-sided colitis Continuous Mucous
Pancolitis Continuous Mucous
Proctosigmoiditis Continuous Mucous
Proctitis Patchy Mucous
Left-sided colitis Patchy Mucous
Pancolitis Patchy Mucous

4.1 Ulcerative Colitis

Continuous inflammation of the colon, starting in the rectum. Symptoms include diarrhea, rectal bleeding, urgency, and abdominal pain. Extraintestinal manifestations include arthritis, skin lesions, and uveitis.

4.2 Crohn’s Disease

Transmural inflammation of any GI tract segment. Presents with abdominal pain, diarrhea, weight loss, and perianal disease. Complications include strictures, fistulas, and abscesses.

4.3 Complications

Toxic megacolon, perforation, strictures, fistulas, malnutrition, and increased cancer risk (especially in UC).

5. INVESTIGATIONS

Diagnostic workup includes endoscopy, imaging, and laboratory tests to confirm IBD and differentiate from other conditions.

Table 337-5: Diagnostic Criteria for IBD

Criteria UC CD
Location Continuous rectal involvement Any GI tract segment
Behavior Continuous inflammation Transmural inflammation
Endoscopic appearance Continuous mucosal inflammation Skip lesions, strictures
Criteria UC CD
Histology Cryptitis, crypt abscesses Granulomas, transmural inflammation

5.1 Endoscopic Findings

UC shows continuous mucosal inflammation with pseudopolyps and ulceration. CD presents with transmural inflammation, strictures, and fistulas.

5.2 Imaging

CT/MRI for CD to assess bowel wall thickening, strictures, and fistulas. Barium enema for UC to show continuous inflammation.

5.3 Laboratory Tests

Elevated CRP/ESR, anemia, and electrolyte imbalances. Calprotectin in stool helps differentiate IBD from infection.

6. MANAGEMENT

Treatment is tailored to disease severity, location, and complications. Includes medical therapy, biologics, and surgery for refractory cases.

Table 337-6: 5-ASA Preparations

Preparation Formulation Delivery Dosing
Sulfasalazine Sulfapyridine-5-ASA Colon 3-6g (acute), 2-4g (maintenance)
Balsalazide Aminobenzoyl-b-alanine–5 -ASA Colon 6.75-9g
Mesalamine (Delzicol) Ethylcellulose microgranules Distal ileum-colon 2.4-4.8g (acute), 1.6-4.8g (maintenance)
Mesalamine (Lialda) Multi-Matrix System Ileum-colon 2.4-4.8g

6.1 Medical Therapy

5-ASA for mild-to-moderate UC. Corticosteroids for acute flares. Immunosuppressants (azathioprine, methotrexate) for maintenance. Biologics (anti-TNF, IL-12/23 inhibitors) for refractory disease.

6.2 Biologic Therapies

Anti-TNF agents (infliximab, adalimumab) are first-line for moderate-to-severe IBD. IL-12/23 inhibitors (ustekinumab) and integrin inhibitors (vedolizumab) are alternatives. JAK inhibitors (tofacitinib) are used for refractory cases.

6.3 Surgical Indications

Total proctocolectomy with ileostomy for UC with dysplasia/cancer. Resection for CD with strictures/fistulas. IPAA for selected UC patients.

7. SPECIAL CONSIDERATIONS

Management of IBD in pregnancy, cancer risk, and drug interactions require careful planning.

Table 337-7: Extraintestinal Manifestations

Category Clinical Course Treatment
Rheumatologic Disorders Peripheral arthritis (asymmetric, migratory) Reduce bowel inflammation
Ankylosing Spondylitis Spinal involvement, HLA-B27 positive Physical therapy, steroids, anti-TNF
Ocular Disorders Uveitis, episcleritis Topical steroids, systemic steroids
Hepatobiliary Disorders Primary sclerosing cholangitis (PSC) Cholecystectomy, ERCP, antifibrotic agents

7.1 Pregnancy

Quiescent IBD has normal fertility. Avoid MTX and anti-TNF agents in first trimester. Use corticosteroids and 5-ASA safely. Cesarean delivery may be needed for anorectal disease.

7.2 Cancer Risk

UC patients with >10 years of disease have 1.5-2x higher CRC risk. CD patients with extensive disease have increased risk of colorectal and small bowel cancer. Endoscopic surveillance recommended.

7.3 Drug Interactions

Anti-TNF agents increase infection risk. JAK inhibitors and biologics may increase cancer risk. Avoid corticosteroids in elderly patients.