Inflammatory Disorders

Question 0

Severe Combined Immunodeficiency

Three defects

Answer 0

Cytokine receptor defect
Adenosine deaminase defect (toxic to lymphocytes)
MHCII deficiency (no cd4 activation)

Humoral and cellular deficiency

Question 1

Digeorge’s syndrome cause and etiology

Answer 1

21q11 microdeletion

Leads to lack of thymus (and no T cells)
Hypocalemia leads to no parathyroid hormone

Question 2

X Linked Agammaglobulinemia

Cause

Answer 2

B cells can’t mature into plasma

Therefore can’t secrete immunoglobulin

Question 3

X Linked Agammaglobulinemia
Due to mutation in
Main etiology

Answer 3

Bruton tyrosine kinase

Recurrent bacterial infection (IgG)
Enterovirus (igA)
Giardia(igA)

Question 4

Most common immunodeficiency

Answer 4

IgA deficiency

Mucosal infections

Question 5

Hyper IgM cause

Answer 5

Mutation in CD40/L receptor
Therefore no second signal and no class switch to IgA,G,E
IgM develops independent of cd4 T cells and class switching

Question 6

Wiskott-Aldrich syndrome

3 symptoms

Answer 6

Thrombocytopenia
Eczema
Recurrent infections (cellular and humoral defect)

Question 7

C5 to C9 deficiency causes what kind of infection

Answer 7

Neiserria

Question 8

C1 inhibitor deficiency

Cause and etiology

Answer 8

Over activation of complement leading to vasodilation and increased permeability

ANGIOEDEMA of skin and mucosal surface

Question 9

SLE is what type(s) hypersensitivity?

Answer 9

II (cytotoxic)

III (AbAg complex)

Question 10

Most common cause of death for SLE

Answer 10

Diffuse proliferative glomerulonephritis

Question 11

Diffuse proliferative is nephritic or nephrotic?

Where are the deposits ?

Answer 11

Both

Deposits of Ag-Ab in sub endothelium between endothelium and basement membrane

Question 12

Libman-sacks endocarditis caused by what?
Where are deposits?
What kind of deposits?

Answer 12

SLE

vegetation on both sides of valves

Non-infectious immune complex deposits

Question 13

SLE
screening positive agent
Diagnosis positive agent

Answer 13

Screening: ANA (anti neutrophil antibody)

Diagnosis: anti dsDNA

Question 14

Can cause drug induced SLE

Answer 14

Hydralazine (smooth muscle relaxant to treat hypertension)

Procainamide

Isoniazide (treats TB)

Common: anti-histone antibody

Question 15

Causes false positive for syphillis test

Answer 15

Anticardiolipin antibody
Found in anti phospholipid syndrome

Which occurs with SLE

Question 16

Hypercoaguability caused by what autoimmune disease?

Answer 16

SLE

Especially susceptzble to
DVT, hepatic vein, placental

Question 17

Autoimmune disease targeting lacrimal and salivary glands

Answer 17

Sjogren syndrome

Question 18

Sjogren syndrome is what type hypersensitivity?

Answer 18

IV lymphocyte mediated

Question 19

Sjogren clinical presentation

Answer 19

Dry eyes dry mouth

Question 20

Sjogren lab findings

Answer 20

ANA,

Anti-ribonucleoprotein Ab
Aka anti-ss-A or B

Question 21

Sjogren syndrome has increased risk for

Answer 21

B cell lymphoma

Presents with UNILATERAL growth of parotid gland

Question 22

Autoimmune disease resulting in activation of fibroblasts

Answer 22

Scleroderma

Question 23

Antibodies present in scleroderma

Answer 23

ANA and

Anti DNA topoisomerase I (aka scl-70)

Question 24

Localized form of scleroderma affects

Answer 24

Calcinosis/antiCentromere
Raynaud syndrome (discoloration)
Esophageal dysmotility
Sclerodactyly (finger fibrosis)
Telangiectasis of skin (dilated blood vessels)