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Questions and Answers
Which cytokine is considered the most potent anti-inflammatory cytokine in the body?
Which cytokine is considered the most potent anti-inflammatory cytokine in the body?
Crohn's disease is characterized by a decrease in gut bacteria like Faecalibacterium.
Crohn's disease is characterized by a decrease in gut bacteria like Faecalibacterium.
True
What genetic defect is associated with early onset childhood inflammatory bowel disease?
What genetic defect is associated with early onset childhood inflammatory bowel disease?
Defect in IL-10 or IL-10 receptor function
The gene polymorphism associated with Crohn's disease is found on chromosome ______.
The gene polymorphism associated with Crohn's disease is found on chromosome ______.
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Match the following components with their respective roles:
Match the following components with their respective roles:
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Which demographic is associated with a higher risk of Crohn's disease compared to ulcerative colitis?
Which demographic is associated with a higher risk of Crohn's disease compared to ulcerative colitis?
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Smoking is protective against Crohn's disease.
Smoking is protective against Crohn's disease.
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What is the impact of appendicectomy on ulcerative colitis?
What is the impact of appendicectomy on ulcerative colitis?
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The presence of microvilli in the colon's crypts contributes primarily to ______ absorption.
The presence of microvilli in the colon's crypts contributes primarily to ______ absorption.
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Match the features of ulcerative colitis (UC) and Crohn's disease (CD):
Match the features of ulcerative colitis (UC) and Crohn's disease (CD):
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Which genetic syndrome is most commonly associated with ulcerative colitis (uc) in childhood?
Which genetic syndrome is most commonly associated with ulcerative colitis (uc) in childhood?
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The Wiskott-Aldrich Syndrome is caused by an autosomal dominant gene mutation.
The Wiskott-Aldrich Syndrome is caused by an autosomal dominant gene mutation.
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What is the primary defect causing IPEX syndrome?
What is the primary defect causing IPEX syndrome?
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Hermansky-Pudlak Syndrome is characterized by interstitial lung disease and __________.
Hermansky-Pudlak Syndrome is characterized by interstitial lung disease and __________.
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Match the following syndromes with their associated features:
Match the following syndromes with their associated features:
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What is a consequence of transmural involvement in gastroenterology?
What is a consequence of transmural involvement in gastroenterology?
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Non-caseating granulomas can form solely within the intestine.
Non-caseating granulomas can form solely within the intestine.
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What type of inflammation occurs in the crypts according to the findings?
What type of inflammation occurs in the crypts according to the findings?
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The presence of _____ granulomas is noted in findings related to Crohn's disease.
The presence of _____ granulomas is noted in findings related to Crohn's disease.
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Match the histological findings with their descriptions:
Match the histological findings with their descriptions:
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Which of the following is considered an earliest finding in diagnosing relapse in remission patients with inflammatory bowel disease?
Which of the following is considered an earliest finding in diagnosing relapse in remission patients with inflammatory bowel disease?
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Cobblestone appearance is a feature associated with Crohn's disease.
Cobblestone appearance is a feature associated with Crohn's disease.
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What are the typical features of the ulcers seen in Crohn's Disease?
What are the typical features of the ulcers seen in Crohn's Disease?
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The condition known for transmural involvement and skip lesions is called ______.
The condition known for transmural involvement and skip lesions is called ______.
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Match the features with their respective conditions:
Match the features with their respective conditions:
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Which feature is more common in Crohn's Disease compared to Intestinal Tuberculosis?
Which feature is more common in Crohn's Disease compared to Intestinal Tuberculosis?
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Intestinal Tuberculosis always involves more than four segments of the colon.
Intestinal Tuberculosis always involves more than four segments of the colon.
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What is a key characteristic of the ileocecal valve (ICV) in Intestinal Tuberculosis?
What is a key characteristic of the ileocecal valve (ICV) in Intestinal Tuberculosis?
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Crohn's Disease primarily affects the terminal ileum and _____ segments of the colon.
Crohn's Disease primarily affects the terminal ileum and _____ segments of the colon.
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Match the following features with the corresponding condition:
Match the following features with the corresponding condition:
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What percentage of ulcerative colitis patients have pancolitis involving the rectum, sigmoid, and colon?
What percentage of ulcerative colitis patients have pancolitis involving the rectum, sigmoid, and colon?
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Ulcerative colitis commonly shows skip lesions throughout the colon.
Ulcerative colitis commonly shows skip lesions throughout the colon.
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What is the first change observed in the mucosa of a patient with ulcerative colitis?
What is the first change observed in the mucosa of a patient with ulcerative colitis?
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The appearance of the colon in chronic long-standing ulcerative colitis can be described as a '______ pipe appearance.'
The appearance of the colon in chronic long-standing ulcerative colitis can be described as a '______ pipe appearance.'
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What is characterized by mucosal ulcers with surrounding translucent edema in the context of inflammatory bowel disease?
What is characterized by mucosal ulcers with surrounding translucent edema in the context of inflammatory bowel disease?
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Match the following changes in ulcerative colitis with their descriptions:
Match the following changes in ulcerative colitis with their descriptions:
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CT scanning is the preferred method for diagnosing inflammatory bowel disease.
CT scanning is the preferred method for diagnosing inflammatory bowel disease.
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Name one appearance associated with inflammatory bowel disease as mentioned in the document.
Name one appearance associated with inflammatory bowel disease as mentioned in the document.
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The ______ sign implies hypervascular mesentery and is typically associated with Crohn's disease.
The ______ sign implies hypervascular mesentery and is typically associated with Crohn's disease.
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Match the imaging findings with their description:
Match the imaging findings with their description:
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Which of the following is NOT a finding typically associated with Crohn's disease in a barium enema?
Which of the following is NOT a finding typically associated with Crohn's disease in a barium enema?
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Toxic megacolon is characterized by extreme dilation of the colon.
Toxic megacolon is characterized by extreme dilation of the colon.
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What is the most common type of Crohn's disease indicated in a barium enema?
What is the most common type of Crohn's disease indicated in a barium enema?
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The appearance of the colon that is often associated with inflammation or strictures is referred to as the ______ appearance.
The appearance of the colon that is often associated with inflammation or strictures is referred to as the ______ appearance.
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Match the following findings from a barium enema with their descriptions:
Match the following findings from a barium enema with their descriptions:
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What symptom is commonly relieved by diarrhea in patients with Crohn's disease?
What symptom is commonly relieved by diarrhea in patients with Crohn's disease?
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Fistulas are abnormal connections that can occur due to Crohn's disease.
Fistulas are abnormal connections that can occur due to Crohn's disease.
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Name one systemic symptom associated with Crohn's disease.
Name one systemic symptom associated with Crohn's disease.
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What symptom is most commonly associated with proctitis in ulcerative colitis?
What symptom is most commonly associated with proctitis in ulcerative colitis?
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Proctocolitis is characterized by small, frequent stools that may contain blood, pus, and mucus.
Proctocolitis is characterized by small, frequent stools that may contain blood, pus, and mucus.
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What is the most sensitive biomarker used to assess the severity of ulcerative colitis?
What is the most sensitive biomarker used to assess the severity of ulcerative colitis?
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The endoscopic appearance of ulcerative colitis is characterized by a red, velvety mucosa with decreased vascularity and ______.
The endoscopic appearance of ulcerative colitis is characterized by a red, velvety mucosa with decreased vascularity and ______.
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Match the following clinical features with their corresponding ulcerative colitis types:
Match the following clinical features with their corresponding ulcerative colitis types:
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Study Notes
Management of Inflammatory Bowel Disease
- Supportive care and replacement therapy
- Gene therapy
Investigations
- Genetic testing
Markers of T-reg cells
- CD3: Pan T-cell marker
- CD25: T-reg cell
- CD4: Helper T-cell
Early Onset Childhood IBD
- Defect in IL-10 or IL-10 receptor function
- Severe refractory IBD in early life
Pathogenesis of IBD
- Exogenous and endogenous factors → Dysregulated mucosal immune response in a genetically susceptible individual.
- Most potent anti-inflammatory cytokine in the body: IL-10
Ulcerative Colitis
- Pathogenesis is unclear.
- Overactive adaptive immune system: Th lineage.
Crohn's Disease
- Genetically susceptible individuals:
- NOD2 (CARD15) gene polymorphism on chromosome 16.
- Dysregulated mucosal immune response:
- Defective innate immunity:
- Decreased clearance of intracellular organisms (mycobacterium paratuberculosis, m.avium complex)
- Decreased defensin production from Paneth cells
- Defective autophagy
- Defective innate immunity:
- Overactive adaptive immune system (Th lineage: Crohn's disease).
- Decrease in gut bacteria like Faecalibacterium (Gut protective).
Epidemiology
- Overall:
- CD > UC
- Males > Females
Comparison of UC and CD
-
Female : Male Ratio:
- UC: No information available
- CD: No information available
-
Age:
- UC: 2 peaks: 15-30 years & 70-90 years
- CD: No information available
-
Ethnicity:
- UC: Jews > Non-Jewish white > African American > Hispanic > Asian
- CD: No information available
-
Smoking:
- UC: Protective
- CD: Causative
-
Appendicectomy:
- UC: Protective
- CD: No association
-
OCP:
- UC: No increased risk
- CD: Increased risk
-
Familial Inheritance (Concordance):
- UC: No information available
- CD: Significant CD > UC
-
Antibiotics in 1st year of life:
- UC: No information available
- CD: Increases risk of IBD by 3 times
Behçet's disease
- GI manifestations like Crohn's disease.
- Share a common antibody: ASCA (Anti-Saccharomyces cerevisiae antibody).
Prolonged use of α-methyl dopa
- Causes features similar to ulcerative colitis.
Campylobacter jejuni
- Risk factor for IBD
- Also causes large bowel diarrhea, Guillain Barre syndrome.
Mucosa
- Villi are absent.
- Crypts: Contain microvilli - water absorption.
- Goblet cells: Secrete mucus.
- Diarrhea > 200 ml of stool volume.
Genetic Syndromes Associated With IBD
- Present as IBD in childhood:
Genetic Syndromes - UC (Non-granulomatous)
- Turner's syndrome: m/c
Genetic Syndromes - CD (Granulomatous)
- Wiskott-Aldrich Syndrome
- von Gierke's disease
- Hermansky-Pudlak syndrome
Clinical Features of Genetic Syndromes
Syndrome | Defect/Inheritance | Triad of: | Features |
---|---|---|---|
Wiskott-Aldrich Syndrome | WASP gene mutation (x-linked recessive) | 1. Recurrent sino-pulmonary infections. 2. Recurrent eczema. 3. Recurrent thrombocytopenia (micro platelets). | Platelet dysfunction with normal platelet count. |
Hermansky-Pudlak Syndrome | Autosomal recessive | Interstitial lung disease: Pulmonary fibrosis. Oculocutaneous albinism. Hypoglycemia +/- Seizures, Doll facies. | |
von Gierke's Disease | Glucose-6-phosphatase deficiency | Hepatomegaly. Lactic acidosis. Hyperuricemia. Hyperlipidemia. |
IMMUNE POLYENDOCRINOPATHY X-LINKED SYNDROME (IPEX)
- Physiology: FOXP3: Transcription factor → Normal T-reg cell function (Cell tolerance)
- Pathology: Loss of FOXP3 function → Reduced cell tolerance → IPEX syndrome.
- Triad of:
- Endocrinopathy (Type 1 Dm / Thyroiditis)
- Dermatitis
- uc-like enteropathy
Microscopy
- Transmural involvement: Involvement extends up to the muscularis propria, potentially leading to fissures and fistulas.
- Serosa involvement: This is a specific type of transmural involvement affecting the serosa layer.
- Non-caseating granulomas: Granulomas are present at various levels and can even form outside the intestine.
- Changes in crypts:
- Cryptitis: Inflammation of the crypts
- Crypt abscesses: Abscesses (pus-filled pockets) within the crypts.
- Crypt architecture: The structure of the crypts appears normal.
- Pyloric metaplasia: A change in the pyloric region of the intestine.
- Lymphoid aggregates: Clusters of lymphoid tissue.
- Possible causes of non-caseating granulomas:
- Sarcoidosis
- Tuberculosis
- Cat Scratch disease
- Berylliosis
- Hypersensitivity pneumonitis
- Crohn's disease
Crohn's Disease vs Intestinal Tuberculosis
- Involvement of ICV:
- Crohn's disease: Common
- Intestinal Tuberculosis: Usually strictured and patulous ICV
- Involvement of colon:
- Crohn's disease: ≥4 segments, especially terminal ileum and ICV
- Intestinal Tuberculosis: No information available.
Microscopy - Ulcerative Colitis
- Superficial Involvement: mucosa & superficial part of submucosa
- Cryptitis with loss of crypt architecture
- Paneth cell metaplasia (Seen distal to hepatic flexure)
- Basal plasma cell and lymphoid aggregates
- Earliest finding
- Can help in diagnosing relapse in remission patients
Factors against the diagnosis of ulcerative colitis
- Transmural involvement
- Skip lesions
- Deep ulcers
Pathology: Crohn's Disease
- Site of Involvement:
- Ileocolitis: 40-50%
- Ileitis: 30-40%
- Colitis: 15-20%
Macroscopy - Crohn's Disease
- Deep, transmural, asymmetric skip lesions
- Earliest finding: Aphthous ulcers → Small, translucent ulcers
- Most visible finding: Linear, Serpenginous ulcers
- Cobblestone appearance: The ulcers fuse longitudinally and transversely with areas of uninvolved mucosa in between
- Peri rectal fissures, fistulas and abscesses
- Fistula tracts resolve by fibrosis
- a.Thickening of the wall
- b.Strictures
- Fistula tracts resolve by fibrosis
Garden Hose Appearance
- Tubular lumen with extensive transmural fibrosis and stenosis seen in terminal ileum
- Creeping fat: Thickened mesentery encasing the bowel wall
- Note: Garden hose appearance in esophagus → well developed progressive systemic sclerosis
Pathology: Ulcerative Colitis
- Site of Involvement:
- Rectum alone: 50%
- Extension to sigmoid colon: 30-40%
- Pancolitis (Rectum, sigmoid, colon involved): 10-20%
- 10-20% of pancolitis patients: Ileum involvement → Backwash ileitis
Macroscopy - Ulcerative Colitis
- Superficial, continuous, symmetrical lesions
- Continuous involvement: Rectum → Sigmoid → Colon → Ileum (No skip lesions)
- 1st Change: Erythematous mucosa with fine, granular surface
- Hemorrhagic, edematous, ulcerated mucosa
- Pseudopolyps: Inflammatory polyp d/t epithelial regeneration
Chronic Long-Standing UC (rare)
- Lead pipe appearance (Atrophic, featureless colon):
- a.Loss of haustrations
- b.Shortening and narrowing of colon
- Fulminant colitis (very rare): Toxic megacolon (Colon diameter >6mm).
Additional Information
- Haustrations:
- Present (uninvolved colon)
- Absent
- Lead pipe appearance: Seen on barium study
Barium Enema Radiology
- Normal mucosa: A healthy lining of the colon.
- Fine mucosal granularities: Tiny, granular bumps on the mucosal layer.
- Superficial ulcerations: Shallow sores affecting the surface of the lining.
- Extensive ulcerations: Deeper sores affecting more of the lining.
- Inflammatory pseudopolyps: Small growths caused by inflammation.
- Deep ulcers extending to submucosa: Deep sores that penetrate into the submucosal layer.
- Collar stud ulcers: Ulcers with a characteristic collar-like appearance.
- Lead pipe appearance: Abnormally uniform, straight appearance of the colon (often associated with inflammation/strictures).
- Toxic megacolon: Extreme dilation of the colon.
Crohn's Disease
- Clinical Features:
- Ileocolitis (most common):
- Episodic right lower quadrant pain.
- Relieved with diarrhea (Large bowel diarrhea d/t colitis).
- D/t Th, profile (IL-2, IFN Y):
- Fever.
- Weight loss.
- Systemic symptoms.
- Jejunoileitis: Malabsorption due to inflammation of the jejunum and ileum.
- Palpable inflammatory mass: Thickening of the bowel wall due to inflammation.
- Bowel obstruction: Blockage of the bowel.
- Fistulas: Abnormal connections between the bowel and other structures (Enterovesical > Perianal).
- Fissures: Cracks in the bowel lining.
- Fibroses & strictures: Scar tissue and narrowings in the bowel.
- Ileocolitis (most common):
Additional Notes - Crohn's Disease
- Earliest change: Fine mucosal granularities.
- Serrations producing hazy margin.
- Mild narrowing of lumen.
- Loss of rectal valves.
- Backwash ileitis (Inflammation of terminal ileum).
Ulcerative Colitis - Clinical Features
- Based on the level of involvement:
Proctitis
- Painless bleeding PR (most common)
- Differential Diagnosis (D/d):
- Hemorrhoids
- Malignancies
- Differential Diagnosis (D/d):
- Slow proximal transit → Constipation
- Normal physical examination.
- Systemic symptoms: very rare (Th2 lineage → Involves IL-4, 5, 13).
- 90% have relapsing course.
Proctosigmoiditis
- Streaks of blood onto formed stool.
Proctocolitis
- Large bowel diarrhea:
- 1.Small, frequent stools with blood, pus, and mucus.
- 2.Tenesmus
Fecal calprotectin
- most sensitive biomarker.
- used to assess disease severity, response to Rx & to diagnose relapse.
Endoscopy - Ulcerative Colitis
- Symmetrical, superficial, and continuous involvement.
- Findings:
- Erythematous, red, velvety mucosa (earliest).
- ↓sed vascularity → mucosal ulcerations with fine granulations and friability (Mayo score)
- Lead pipe/pipestem colon (very rare in endoscopy).
Radiology - Barium Enema - Ulcerative Colitis
- Target Sign: Characterized by mucosal ulcers with surrounding translucent edema.
- Significance: Earliest radiologic and endoscopic finding for IBD.
CT Scan - IBD
- Limited Role: CT scanning plays a limited role in diagnosing IBD, with MR enterography being preferred.
- Possible findings:
- String sign: Indicates a fibrosed bowel.
- Sinus tracts: Visible on the image as a tract or channel.
- Fissures and string sign: Indicate specific pathologies.
- Target appearance: Presence of thickened colonic walls.
- Comb sign: A sign typically associated with Crohn's disease, implying hypervascular mesentery.
- Fat halo sign: Describes a particular appearance.
- Tissue Attenuation: The inner and outer layers of tissues show soft tissue attenuation, while the middle layer displays fatty attenuation.
Other Appearances - CT Scan - IBD
- Longitudinal ulceration
- Cobblestone appearance
- Rose thorn (Transmural ulcers)
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Description
Test your knowledge on the management and pathogenesis of Inflammatory Bowel Disease (IBD). The quiz covers supportive care, genetic factors, and the immune markers associated with both ulcerative colitis and Crohn's disease. Assess your understanding of IBD's complexities and therapeutic approaches.