Celiac disease

Question 1

Celiac disease is an ______ disease characterized by chronic intestinal inflammation and varying degrees of intestinal mucosal atrophy and malabsorption

Answer 1

autoimmune

Question 2

Describe the classic presentation of celiac disease

Answer 2

weight loss, watery diarrhea, and steatorrhea with a tympanitic, doughy abdomen on exam

only 10% of patients

Question 3

List non-classical or other signs of celiac

Answer 3

weight gain and obesity
gastroesophageal reflux disease
irritable bowel syndrome with abdominal pain and constipation,
pancreatitis
myocarditis,
aphthous ulcers of the oral mucosa
lymphocytic and collagenous colitis
hyposplenism
asymptomatic IgA nephropathy

Question 4

What factors constitute “minimal disease” in celiac?

Answer 4

iron and folate deficiency
osteogenic bone disease- Ca and Vitamin D deficiency
Bloating and cramping only

Question 5

List factors that constitute severe disease in celiac

Answer 5

Nutritional deficiencies
megaloblastic anemia
neurologic disease (B12)
profound diarrhea
infertility 
weight loss

Question 6

Celiac is an immunologic reaction to the gluten protein in wheat called _____ or structurally similar proteins in rye and barley

Answer 6

gliadin

Question 7

The most severe involvement in celiac disease is in the ________ where fragments of indigestible peptides are at highest concentration

Answer 7

upper small bowel, beginning in duodenum

Question 8

Celiac disease is driven by _________ in the small bowel, a population of intraepithelial lymphocytes

Answer 8

gamma delta T cells

Question 9

Describe the pathologic molecular mimicry in celiac disease

Answer 9

Alpha-gliadin is similar to the E1b
protein of the human adenovirus serotype 12 (Ad12).
Ad12 is usually found in the human digestive tract. The E1b protein causes inflammation in celiac patients.

Question 10

List the triad of factors that leads to celiac disease

Answer 10

environmental trigger- alpha gliadin similar to E1b of Ad12

genetic predisposition- DQ2, DQ8

leaky gut- zonuin increases intestinal permeability

Question 11

How does genetic predisposition contribute to celiac pathophysiology?

Answer 11

Patients have genes for HLA-DQ2 or DQ8 on APCs

tTG binds to DQ2/DQ8 and presents to T cells which mount an immune response

Question 12

How do patients with celiac disease develop a leaky gut?

Answer 12

The tight junctions of the small intestine’s epithelium can be made more permeable through the action of the protein zonulin, which is secreted by the epithelial lining

In normal circumstances, zonulin allows for trafficking of immune cells, fluid, and large proteins.
The zonulin protein is mimicked by proteins manufactured by certain bacteria

Question 13

What is the function of zonulin in normal circumstances?

Answer 13

allows for trafficking of immune cells, fluid, and large proteins.

Question 14

_____ is mimicked by proteins manufactured by some bacterial, so people with a genetic predisposition can develop celiac disease when exposed to environmental bacterial triggers

Answer 14

zonulin

Question 15

List antibody markers associated with celiac disease

Answer 15

best: anti-tTG IgA
others: anti-gliadin IgG/IgA

Question 16

___ deficiency is present in 2% of celiac patients and can make some diagnostic tests falsely negative

Answer 16

IgA

Question 17

__________ are no considered not just an epiphenomenon but play a role in disease process

Answer 17

tTG antibodies

Question 18

Describe the histologic findings of duodenal tissue biopsy in celiac disease

Answer 18

villus atrophy
crypt hyperplasia
mucosal inflammation
increased intraepithelial lymphocytes

Question 19

False negative biopsy results will be obtained if a patient adopts __________ prior to biopsy

Answer 19

gluten free diet

Question 20

List conditions on the differential diagnosis for celiac

Answer 20

infectious gastroenteritis or bacterial overgrowth
eosinophilic enteritis
HIV
lymphoma
Zollinger-Ellison syndrome
ischemic or radiation damage
starvation

Question 21

The mainstay of treatment of celiac disease is:

Answer 21

gluten free diet

must avoid wheat, barely, rye

Question 22

List complications of full blown celiac disease

Answer 22

osteoporosis, short stature, chronic anemia, microscopic colitis, pancreatic insufficiency, lactose intolerance

Question 23

In celiac, selection for certain clones of immune cells may predispose for transformation to _______

Answer 23

lymphoma

Question 24

_________ is a severe form of celiac that is not responsive to diet change and would be concerning for possibly progressing to or masking lymphoma

Answer 24

ulcerative jejunoileitis

Question 25

_______ does not respond to gluten free diet and is treated with steroids or immunosuppressants

Answer 25

refractory sprue

Question 26

The neurologic manifestations of celiac disease can have the same MRI appearance as ____

Answer 26

ALS

Question 27

________ is a pruritic papulovesicular rash seen on the extensor surfaces of the extremities

Answer 27

dermatitis herpetiformis

Question 28

Describe quantitatively the relationship between dermatitis herpetiformis and celiac disease

Answer 28

80% of patients with DH have celiac disease.

10% of celiac patients have DH.

Question 29

______ is a topical agent that can be used to treat the lesions of dermatitis herpetiformis

Answer 29

dapsone