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Block D - Digestion and Metabolism > Pathology -- Autoimmune Diseases > Flashcards

Pathology -- Autoimmune Diseases Flashcards

1
Q

Define MALT

A

Mucosa Associated Lymphoid Tissue = a specialized immune system which protects mucosal surfaces (i.e. GIT, bronchial tree, nasopharynx, GU tract)

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2
Q

Role of MALT in the GIT

A
  • Absorption of nutrients
  • Need and effective barrier and a selective response (innate and acquired immune system) to various substances (harmless vs. harmful)
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3
Q

What constitutes an effective barrier in the mucosal immune system

A

Intact intestinal epithelium (surface mucosa, peristalsis, protective secretory factors)

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4
Q

Define the innate immune system

A

Initial response to antigen exposure (neutrophils, macrophages, NKCs)

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5
Q

Define the adaptive immune system

A

Specific immuntiy to antigens (APCs with molecules of the major histocompatibility complex) = self from non-self

i.e. B and T lymphocytes, dendritic cells

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6
Q

One hypothetical equation to explain the cause of IBD

A
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7
Q

5 risk factors for IBD

A
  • Age = young
  • Ethnicity = Causacians (particularly Ashkenazi Jews)
  • Family history
  • Geography = US and Europe
  • Smoking
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8
Q

Incidence of IBD

A

CD = 16.9 per 100,000

UC = 12.9 per 100,000

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9
Q

Provinces with the highest incidence of IBD

A

QC and NS

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10
Q

Prevalence of IBD in Canada

A

0.7% (233,000)

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11
Q

Describe the role of family history in the risk of developing IBD

A

1st degree relative = 12 - 25% risk

Monozygotic twins = 60% concordance

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12
Q

Describe the role of smoking in the risk of developing IBD

A

Active smokers are more than 2 times as likely to develop CD than nonsmokers, but less likely to develop UC (smoking is actually protective??)

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13
Q

How is IBD diagnosed?

A

Clinical diagnoses relying on:

  • Clinical history (symptoms)
  • Laboratory findings
  • Endoscopic features
  • Histological evaluation
  • Radiographic evaluation
  • Rulling out infectious etiologies
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14
Q

4 lab tests for IBD

A
  • Tests for anemia
  • B12 deficiency
  • Increased CRP
  • Fecal calprotectin
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15
Q

3 endoscopic methods for diagnosing IBD

A
  • Gastroscopy
  • Colonoscopy
  • Capsule endoscopy
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16
Q

3 radiographic evaluation tools for diagnosing IBD

A

SBFT or CT or MR enterography

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17
Q

2 subsets of IBD

A

Crohn’s disease

Ulcerative colitis

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18
Q

7 characteristics of Crohn’s disease

A
  • Chronic inflammatory disease
  • Affects any segment of the luminal GIT (mouth -> anus)
  • Transmural involvement
  • Rectum usually spared
  • Sharply delineated areas affected with intervening normal bowel (“skip areas”)
  • Noncaseating granulomas
  • Fistulization
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19
Q

Describe the anatomical distribution of CD

A
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20
Q

4 clinical patterns of CD

A

+ Fistulae and abscesses

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21
Q

2 general clinical features of CD

A
  • Intermittent diarrhea and abdominal pain
  • GI bleeding
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22
Q

3 chronic clinical features of CD

A
  • Strictures
  • Fistulas
  • Malabsorption
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23
Q

7 morphological features of CD

A
  • Mucosal erythema/edema
  • Superficial ulcers
  • Deep linear ulcers on axis
  • Nodularity from skip areas (cobblestoning)
  • Bowel wall thickens, lumen narrows (string sign)
  • Edematous mesentery (creeping fat)
  • Extension of fissuring (fistulae, abscesses, adhesions, perforations)
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24
Q

5 histologic features of CD

A
  • Mucosal inflammation
  • Chronic mucosal damage
  • Ulceration (abrupt)
  • Transmural inflammation
  • Granulomas (10 - 30%)
  • Thickening/fibrosis
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25
Q

Describe the mucosal inflammation of CD

A

Crypt abscesses

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26
Q

3 features of chronic mucosal damage seen histologically in CD

A
  • Glandular distortion
  • Pyloric metaplasia
  • Paneth cell metaplasia
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27
Q

How does CD progress anatomically over time?

A

Location remains stable over time (note that 1/3 have penetrating/stricturing complications)

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28
Q

How common is prolonged remission in CD

A

Only 10% within 10 years

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29
Q

4 complications of CD

A
  • Annual incidence of hospitalization = 20%
  • Steroid dependency = 33%
  • Surgery post diagnosis = 50% within 10 years
  • Post-operative recurrence = 50% within 10 years
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30
Q

7 characteristics of UC

A
  • Chronic inflammatory disease
  • Affects the colorectum (may be whole colon = pancolitis)
  • Generally restricted to the mucosa
  • Rectal involvement
  • Contiguous to varying extent
  • 10% with “backwash ileitis”
  • No granulomas
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31
Q

Describe the therapy for CD

A

Top down therapy:

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32
Q

Describe the anatomic distribution of UC

A
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33
Q

Describe the clinical features of UC

A

A spectrum of effects based on disease progression where:

  • Proctitis = mild
  • Left-sided colitis = moderate
  • Pancolitis = severe
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34
Q

5 mild clinical features of UC

A
  • Intermittent bleeding
  • Fewer stools daily
  • Mucous
  • Tenesmus
  • Constipation
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35
Q

3 moderate clinical features of UC

A
  • More bleeding
  • More daily stools
  • Left-sided cramps
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36
Q

5 severe clinical features of UC

A
  • Significant bleeding
  • Frequent stools
  • Increasing pain
  • Fevers
  • Megacolon
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37
Q

When can toxic megacolon occur?

A
  • Most severe cases of UC
  • Corhn’s colitis
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38
Q

Define toxic megacolon

A

Toxic damage to muscularis propria and neural plexus, negatively affecting neuromuscular function –> colon dilates and becomes gangrenous

39
Q

4 morphologic features of UC

A
  • Mucosal erythema, granularity, friability
  • Broad ulceration (on axis)
  • Regenerating mucosa (pseudopolyps)
  • Progressive mucosal atrophy (flattened surface, no mural thickening)
40
Q

Describe the histology of UC

A
  • Chronic mucosal changes similar to CD
    • Mononuclear infiltrate
    • Crypt abscesses
    • Glandular distortion
  • No granulomas
41
Q

Describe the anatomical progression of UC over time

A
  • Disease location remains stable over time
  • Proximal progression in 5 - 15% over 5 years
  • Distal disease = 20% resolve spontaneously
42
Q

10 year colectomy rate for UC

A

9 - 21% (35% at 20)

43
Q

2 complications of UC

A
  • IPAA (ileal pouch anal anastomosis)
  • Risk of colon cancer
44
Q

4 characteristics of IPAA

A
  • Pouchitis (50% within 5 years)
  • Female infertility
  • Nocturnal incontinence
  • Pouch failure
45
Q

What is the risk of colon cancer in UC patients?

A

0.3 - 0.5% per year increase after 8 - 10 years of disease

46
Q

Describe the treatment for UC

A
47
Q

Extraintestinal manifestations of IBD

A
  • Cholangitis (primary sclerosing cholangitis)
  • Hematologic (anemia, hemolysis, amyloid)
  • Eye (episcleritis, uveitis)
  • Thromboembolism
  • Skin (E. nodosum, pyoderma gengrenosum)
48
Q

4 other extraintestinal manifestations of IBD unrelated to the CHEATS mnemonic

A
  • Kidney stones
  • Gallstones
  • Osteomalacia
  • Pericarditis
49
Q

Define celiac disease

A

T-cell mediated immune disease os the small intestine triggered by gliadin (gluten protein) found in wheat, rye, and barley

50
Q

Describe the genetic factors of celiac disease

A

Chromosome 6

  • HLA DQ2 and DQ8 gene loci
  • >98% in celiac disease, but present in 30% of Caucasians; necessary but not sufficient for disease occurrence
51
Q

Pathogenesis of celiac disease

A
  1. On exposure to gliadin, tissue transglutaminase (TTG) modifies the protein
  2. Immune system cross reacts with the small bowel tissue
  3. Intraepithelial infiltration by CD8+ T cells
  4. Mucosal inflammation, crypt hyperplasia and villous atrophy
52
Q

5 classifications of celiac disease

A
  • Classic or typical celiac disease
  • Atypical celiac sprue
  • Asymptomatic (silent) celiac disease
  • Latent (potential) celiac disease
  • Refractory celiac disease
53
Q

Define classical/typical celiac disease

A

Gluten-sensitive enteropathy found in association with villous atrophy, malabsorption and resolution of symptoms and mucosal lesions on a gluten free diet

54
Q

Define atypical celiac sprue

A

Gluten-sensitive enteropathy with only minor GI symptoms but atypical manifestations, including anemia, osteoporosis, arthritis, neurological symptoms and infertility

55
Q

Define asymptomatic (silent) celiac disease

A

Gluten-sensitive enteropathy found after serological screening in asymptomatic patients

56
Q

3 characteristic symptoms of malabsorption

A
  • Steatorrhea
  • Weight loss
  • Vitamin deficiency
57
Q

Define latent (potential) celiac disease

A

Patients who have normal villous architecture on a gluten-containing diet (but have serological markers positive for celiac disease)

58
Q

Define refractory celiac

A

Symptomatic, severe small intestinal villous atrophy, but does not respond to at least 6 months of a strict gluten-free diet

59
Q

Which type of celiac disease is more common?

A

Silent celiac sprue = 7x more common than symptomatic celiac sprue

60
Q

Prevalence of celiac disease in North America

A

1:100 to 1:130

61
Q

Genetic risk factors for celiac disease

A
  • First degree relatives = 10 - 20% disease prevalence
  • Monozygotic twins = 75% concordance
62
Q

Mean age of presentation for celiac disease

A

45

63
Q

8 clinical features of presentation for celiac disease

A
  • Episodic diarrhea (up to 10x per day; nocturnal or early morning diarrhea is common)
  • Distention
  • Steatorrhea
  • Flatulence
  • Weight loss
  • Vague abdomnal discomfort/fatigue
  • Anemia
  • Recurrent aphthous ulcers
64
Q

6 extraintestinal manifestations of celiac disease

A
  • Anemia (impaired reabsorption)
  • Metabolic bone disease
  • Neuropsychiatric disease
  • Hyposplenism
  • Kidney disease
  • Dermatitis herpatiformis
65
Q

Why might anemia accompany celiac disease?

A

Impaired reabsorption in the small intestine

  • Proximal = iron and folate deficiency
  • Ileum = B12 deficiency
66
Q

Why might metabolic bone disease accompany celiac disease?

A
  • Impaired calcium absorption
  • Vitamin D deficiency
67
Q

Define dermatitis herpetiformis

A

Pruritic papules in grouped arrangements that improve on a gluten free diet

68
Q

How do you histologically confirm the presence of dermatitis herpetiformis?

A

Granular IgA deposits in the subepidermal basement membranes

69
Q

5 serological tests that can be done to diagnose celiac disease

A
  • IgA EMA (endomysial antibodies)
  • IgA tTG (tissue transglutaminase)
  • Anti IgA AGA (anti-gliadin antibodies)
  • Anti-IgG AGA
  • Anti-IgG tTG
70
Q

Gold standard for diagnosing celiac disease

A

IgA EMA (endomysial antibodies)

71
Q

Statistical features of the IgA EMA test

A
  • Sensitivity = 90%
  • Specificity = 99%
  • Reproducibility = 93%
72
Q

Statistical features of the IgA tTG test

A
  • Sensitivity = 93%
  • Specificity = 95%
  • Reproducibility = 83%
73
Q

Advantage of using IgA tTG as a diagnostic test for celiac disease

A

Cheaper and more available than IgA EMA

74
Q

Disadvantage of using IgA tTG to diagnose celiac disease

A

False positives in patients with other auto-immune or liver diseases

75
Q

What serological test can be used to assess patient compliance to a gluten-free diet for treating celiac disease?

A

IgA tTG (should fall to normal or “baseline” within 3 - 6 months)

76
Q

Least reliable serological test for diagnosing celiac disease

A

Anti IgA AGA

77
Q

When should Anti-IgG AGA or Anti-IgG tTG be used to diagnose celiac disease?

A

For patients with IgA deficiency (10% of the population)

78
Q

4 endoscopic findings for celiac disease

A
  • Absent folds
  • Scalloping
  • Mosaicism
  • Fissures
79
Q

How can celiac disease be diagnosed by biopsy?

A

Marsh classification by biopsying in D2 and the duodenal bulb

80
Q

Describe the March Classification in diagnosing celiac disease

A

Histological definitions with 5 stages from STAGE ZERO to STAGE FOUR

81
Q

Stage zero of the marsh classification

A

Preinfiltrative mucosa. Increase in IELs (not enough to diagnose celiac at this stage)

82
Q

Stage one of the marsh classificaiton

A

Infiltration of the lamina propria with lymphocytes

83
Q

Stage two of the marsh classification

A

Crypt hyperplasia

84
Q

Stage three of the marsh classification

A

Villous atrophy

85
Q

Stage four of the marsh classification

A

Total mucosal atrophy. Complete loss of villi, enhanced apoptosis, more crypt hyperplasia

86
Q

How to treat celiac disease

A

Gluten free diet

87
Q

7 advice points to follow a gluten free diet

A
  • Avoid all foods containing wheat, rye, and barley gluten
  • Avoid all oats initially (gluten contamination)
  • Read all labels and study ingredients of processed foods
  • Beware of gluten in medications, food additives, emulsifiers, or stabilizers
  • Limit milk and milk products initially
  • Avoid all beer, lagers, ales, and stouts
  • Wine, liqueurs, ciders, and spirits, including whiskey and brandy are allowed
88
Q

Why is celiac disease associated with lactose intolerance?

A

Loss of villi in small intestine = loss of lactase found on the lining = inability to digest lactose

89
Q

Complications of uncontrolled celiac disease

A

Malignancy (2.6 - 3.1 fold increase risk)

  • Lymphoma (2/3)
  • Oropharynz, esophagus, and small intestine malignancies (1/3)

Ulcerative jejunoileitis

90
Q

How can someone reduce their risk for malignancies if they have celiac disease

A

Risk is reduced to normal after 5 years of gluten-free diet

91
Q

Specific lymphoma associated with celiac disease

A

EATL = enteropathy associated T cell lymphoma

92
Q

Problem with EATL

A

Difficult to diagnose and fatal

93
Q

When can EATL occur?

A

After 20 - 30 years of having uncontrolled celiac disease

Block D - Digestion and Metabolism (29 decks)

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