Immune diseases Flashcards

1
Q

Ankylosing spondylitis

A

HLA B27

90% -100% relative risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Autoimmune diseases HLA

A

HLA-DR

i.e. RA, TI DM has a 4% increased risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

IEM HLA

A

21-hydroxylase deficiency (BW47)

hereditary hemochromatosis HLA-A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Anaphylaxis

A

TI
causes vascular shock, edema, dyspnea

d/t administration of foreign proteins, drugs, food, insect toxins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Myasthenia gravis

A

Type II

Ab against ACh receptors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Graves disease

A

Type II

Ab stimulates TSH-R

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Transfusion reactions

A

Type II

preformed host Abs attack donor cels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

EBF

A

Type II IgG from mother cross placenta and attack fetal blood antigens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

autoimmune hemolytic anemia

A

type II

produce Abs agaisnt their own blood cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Adverse drug reactions

A

Type II

drugs act as a hapten and attach to RBCs to which Abs react

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Goodpasture

A

type II

Abs against IV collagen in the BM of glomeruli and lungs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

acute serum sickness

A

Type III

causes arthritis, vasculitis, nephritis

fibrinoid necrosis of the vessels

deposition of immune complexes d/t introduction of large amount foreign substance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Tuberculin reaction PPD

A

Type IV DTH
previously sensitized individuals
re-exposure causes reddening and induration at site 8-12h post-exposure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

granulomatous infection

A

strong T cell activation to persistent non-degradable antigen (TB)
a. Activates macrophages into epithelioid cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

contact dermatitis

A
  1. Contact with urushiol causes vascular dermatitis

a. Epidermal blistering with mononuclear infiltrates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

MS

A

Type IV
protein antigens in CNS
demyelination with perivascular inflammation, paralysis, ocular lesions

17
Q

AIRE mutation

A

autoimmune polyendocrinopathy

18
Q

IPEX

A
loss of FOXp3 signaling 
No Tregs (peripheral tolerance) 
systemic autoimmune disease
19
Q

ALPS

A

(autoimmune lymphoproliferative syndrome)

mutations in the Fas gene decreases peripheral tolerance

20
Q

SLE dx

A

Type III HS
ANA Abs and dsDNA Abs and Smith antigen

antiphospholipid Abs–>+ syphyllis test,

formation of immune complexes and failure to remove apoptotic bodies

21
Q

SLE sx

A
acute necrotizing vasculitis 
Renal (glomerularnephritis) 
Malar rash (IC deposition at dermo-epidermal junction) 
Joint pain w/o deformity 
Neuropsychiatric (seizures, psychosis)
pericarditis 
nonbacterial verrucous endocarditis (Libman-Sacks)
Pleuritis
Fever and fatigue
hemolytic anemia
22
Q

drug induced Lupus erythematous

A

hydralazine, procainamide, isoniazid, d-penicillamine

anti-histone Ab-s

23
Q

Sjogren syndrome

A

AI destruction of lacrimal and salivary glands
mixed T/B tumor
+rheumatoid factor, antibodies against SSA (ro) and SSB (la)
xerostomia, dry-eyes
increased risk for lymphomas
increased risk for other autoimmune disorders (RA)

24
Q

Systemic sclerosis

A

CD4+ mediated microvascualr damage and narrowing –>fibrosis and widespread damage to blood vessels

DNA topoisomerase Abs

sclerotic atrophy, edema, increased collagen in dermis, thickening of dermis, ulceration, raynauds, GERD, pulmonary HTN, pericarditis, renal HTN and failure

25
Q

CREST

A

limited system sclerosis
calcinosis, raynauds, esophageal dysfunciton, sclerodactylyl, telangiectasia

**anti-centromere Abs

26
Q

inflammatory myopathies

A

AI damage of skeletale mm.

histidyl tRNA synthetase antigen (Jo-I)

27
Q

Mixed CT disease

A

mixture of SLE, SS, polymyositis
renal involvment, corticosteroids

U1 RNP Abs

28
Q

polyarterites Nodosa

A

necrotizing inflammation of walls of blood vessels

non-infective vasculitis

29
Q

RA

A

autoimmune mediated damage of synovium, joints, extra-articular tissue
Rheumatoid nodules can present on other tissue

seroposotive spondyloarthropathy and serositis

30
Q

Goodpasture syndrome

A

type II
Abs against non-collagenous domain of collagen IV

destruction of BM of lungs, renal glomeruli
glomerulonephritis and pneuomonitis

31
Q

x-linked Agammaglobulinemia

A

failure of maturation during b cell development
d/t mutation in Btk gene
begin after 6mo (after maternal Ig end)
recurrent bacterial infections or extracellular virus

absent B cells and Ig, underdeveloped nodes, normal T cells

32
Q

Common variable immunodeficiency

A

Defect in B cell ability to mature into plasma cells
recurrent bacterial infections or extracellular virus
normal B cell levels, absent Ig levels
hyperplastic B cell areas (d/t loss of feedback inhibition)

concordance with autoimmune disease

33
Q

Isolated IgA deficiency

A

low levels of serum and secretory IgA

most common immunodeficiency
defect in mucosal defenses
resp, GI, and UG tract infections

increased risk for asthma and AI disease d/t class switch to IgE

34
Q

Hyper-IgM

A
x linked mutation in the CD40L prevents class switching and affinity maturation of BCRs
normal/high IgM, no IgA, G, or E 

IgM are reactive to blood causing hemolytic anemias, thrombocytopenias, neutropenias

recurrent bacterial infections d/t loss of opsonization

p. jiroveci

35
Q

DiGeorge

A

T cell defect resulting from failure of 3rd and 4th pouchs to form

thymic aplasia–>no T cells–> increased viral and fungal infections
normal-low Igs

loss of parathyroids–>tetany
craniofacial and cardiac defects

36
Q

SCID

A

defects in T cell development –>secondary defects in B cell function

chronic infection, thrush, diaper rash, FTT
maternal cells can cause GVH

treatment= bone marrow transplant or gene therapy

37
Q

Wiskott- Aldrich

A

XLR
TIE= thrombocytopenia,infections, eczema,
depletion of peripheral T cells

Low IgM, normal IgG, High IgA and IgE

38
Q

Kaposi Sarcoma

A

induced by KS herevirus
vascular tumor with spindle shaped cells mixed endothelial and smooth muscle, slit-like vascular spaces

found on face and mouth

39
Q

AIDS related cancers

A

Kaposi sarcoma (KS herpes virus)
systemic lymphomas, CNS lymphomas (EBV)
carcinomas of cervix and anala canal (HPV)