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30 Cards in this Set

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Strongest HLA association with celiac disease
HLA-DQ2 (95% of patients)
Characteristic biopsy findings in celiac disease
blunting of villi with crypt hyperplasia and expansion of lamina propria mononuclear cells
Cutaneous condition associated with celiac disease
Dermatitis herpetiformis--pruritic, vesicular/excoriated lesions on the extensor surfaces of the extremeties, trunk, buttocks, scalp, neck
4 conditions associated with celiac disease
Dermatitis herpetiformis
IgA deficiency
Autoimmune disorders
Malignant disease
What type of Th cells/cytokines are associated with celiac disease?
Th1 cells, producing IFN-gamma and TNF-alpha, which then act on intestinal macrophages to produce proinflammatory il-1beta and TNF-alpha
What is the most common cause of vitamin B12 deficiency?
Autoimmune gastritis (pernicious anemia)
Major immunologic features of autoimmune gastritis?
Autoantibodies to parietal cell and intinsic factor (major targets are H+ and K+ATPase). CD4+ T-cells mediate the disease
What age/gender is primarily affected by pernicious anemia?
Older women
How do you diagnose pernicious anemia?
Schilling's test for the detection of vitamin B12 malabsorption.
Pathophysiology of autoimmune gastritis?
atrophy of gastric parietal cell (mediated by CD4+T-cells) and loss of intrinsic factor production by zygomatic cells, leading to vitamin B12 malabsorption
Treatment of autoimmune gastritis
vitamin b12 injections (lifelong)
Prognosis of untreated autoimmune gastritis
Neurologic damage
Biopsy findings in Crohn's disease
Transmural granulomatous inflammation of the bowel wall
What genetic mutation is most strongly associated with Crohn's disease?
Mutation of NOD2 (20-50% of patients with Crohn's).
What Th-cell type (and cytokines) are associated with Crohn's disease?
Th1, IL-12/INF-gamma
True or false: patients with Crohn's disease mount an inflammatory response to their own bowel flora?
True.
What ethnic group is particularly affected by Ulcerative Colitis?
Ashkenazi Jews
Biopsy findings in ulcerative colitis?
Mucosal inflammation and superficial cryptitis.
What age/gender is typically affected by Type I autoimmune hepatitis?
women of any age
Autoantibodies in type I autoimmune hepatitis?
ANA
ss or ds DNA
p-ANCA
smooth muscle
actin
soluble liver antigen
What age/gender is typically affected by Type II autoimmune hepatitis?
Children, women
5 Conditions associated with type II autoimmune hepatitis?
Type I DM
Arthritis
Autoimmune thyroiditis
Sjogren's syndrome
Vitiligo
What type of autoantibodies is associated with type II autoimmune hepatitis?
Cytochrome P450
What age/gender is tyically affected by Type III autoimmune hepatitis?
Any age (women>men)
What type of autoantibodies are associated with type III autoimmune hepatitis?
Anti-liver-kidney-microsomes
Liver-cytosol-antigen
+/- soluble liver or pancreas Ag
What age/gender is most commonly affected by primary biliary cirrhosis?
Middle aged women (90% of patients)
What type of autoantibodies are associated with primary biliary cirrhosis?
anti-mitochondrial (usually IgG, although IgM or IgA are possible)
What age/gender is primarily affected by primary sclerosing cholangitis?
Male predominance (2:1)
What condition is strongly associated with primary sclerosing cholangitis?
UC (close to 90% of patients with PSC have UC)
What are key pathologic findings in primary sclerosing cholangitis?
Fibrous, obliterative that occurs in a segmental pattern in intrahepatic and extrahepatic bile ducts. CD8 T-cells predominate.