Flashcards in Celiac disease Deck (29):
1
Celiac disease is an ______ disease characterized by chronic intestinal inflammation and varying degrees of intestinal mucosal atrophy and malabsorption
autoimmune
2
Describe the classic presentation of celiac disease
weight loss, watery diarrhea, and steatorrhea with a tympanitic, doughy abdomen on exam
only 10% of patients
3
List non-classical or other signs of celiac
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
4
What factors constitute "minimal disease" in celiac?
iron and folate deficiency
osteogenic bone disease- Ca and Vitamin D deficiency
Bloating and cramping only
5
List factors that constitute severe disease in celiac
Nutritional deficiencies
megaloblastic anemia
neurologic disease (B12)
profound diarrhea
infertility
weight loss
6
Celiac is an immunologic reaction to the gluten protein in wheat called _____ or structurally similar proteins in rye and barley
gliadin
7
The most severe involvement in celiac disease is in the ________ where fragments of indigestible peptides are at highest concentration
upper small bowel, beginning in duodenum
8
Celiac disease is driven by _________ in the small bowel, a population of intraepithelial lymphocytes
gamma delta T cells
9
Describe the pathologic molecular mimicry in celiac disease
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.
10
List the triad of factors that leads to celiac disease
environmental trigger- alpha gliadin similar to E1b of Ad12
genetic predisposition- DQ2, DQ8
leaky gut- zonuin increases intestinal permeability
11
How does genetic predisposition contribute to celiac pathophysiology?
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
12
How do patients with celiac disease develop a leaky gut?
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
13
What is the function of zonulin in normal circumstances?
allows for trafficking of immune cells, fluid, and large proteins.
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
zonulin
15
List antibody markers associated with celiac disease
best: anti-tTG IgA
others: anti-gliadin IgG/IgA
16
___ deficiency is present in 2% of celiac patients and can make some diagnostic tests falsely negative
IgA
17
__________ are no considered not just an epiphenomenon but play a role in disease process
tTG antibodies
18
Describe the histologic findings of duodenal tissue biopsy in celiac disease
villus atrophy
crypt hyperplasia
mucosal inflammation
increased intraepithelial lymphocytes
19
False negative biopsy results will be obtained if a patient adopts __________ prior to biopsy
gluten free diet
20
List conditions on the differential diagnosis for celiac
infectious gastroenteritis or bacterial overgrowth
eosinophilic enteritis
HIV
lymphoma
Zollinger-Ellison syndrome
ischemic or radiation damage
starvation
21
The mainstay of treatment of celiac disease is:
gluten free diet
must avoid wheat, barely, rye
22
List complications of full blown celiac disease
osteoporosis, short stature, chronic anemia, microscopic colitis, pancreatic insufficiency, lactose intolerance
23
In celiac, selection for certain clones of immune cells may predispose for transformation to _______
lymphoma
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
ulcerative jejunoileitis
25
_______ does not respond to gluten free diet and is treated with steroids or immunosuppressants
refractory sprue
26
The neurologic manifestations of celiac disease can have the same MRI appearance as ____
ALS
27
________ is a pruritic papulovesicular rash seen on the extensor surfaces of the extremities
dermatitis herpetiformis
28
Describe quantitatively the relationship between dermatitis herpetiformis and celiac disease
80% of patients with DH have celiac disease.
10% of celiac patients have DH.
29