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Flashcards in Immunopath Doc - Nichols Deck (59):
1

A rapid immunological reaction within minutes after presentation of antigen bound to mast cells is what type of hypersensitivity?

Type 1

2

What are the clinical features /characteristics of anaphylaxis?

Shock, edema, bronchoconstriction

3

Type 2 hypersensitivity is what?

IIa: Antibody-dependent
Complement-mediated
Cytotoxicity or

IIb: Antibody-dependent
Cell-mediated
Cytotoxicity

4

Immune complex-mediated hypersensitivity is what type?

- where is it most damaging?

Type 3

- Blood vessels and glomeruli

5

Cell-mediated hypersensitivity is an immunological reaction caused by

sensitized CD-4 T cells, sometime triggering macrophages response.

6

Anti-cyclic citrullinated peptide (anti-CCP)

R.Arthritis

7

Anti-centromere

CREST syndrome

8

Anti-Jo-1 (one type of anti synthetase)

Polymyositis / dermatomyositis

9

Anti-Scl70 (anti-DNA topoisomerase)

Systemic sclerosis, diffuse

10

Anti-RNA polymerase (anti-U3 RNP)

systemic sclerosis

11

Anti-U1 RNP (anti-RNP)

mixed connective tissue diseases

12

Anti-Smith (anti-Sm)

Lupus

13

Anti-dsDNA

Lupus

14

Anti-nuclear (ANA)

Lupus and other rheumatic diseases

15

Anti-SSA (anti-Ro)

Sjogren syndrome, neonatal lupus, subQ lupus

16

Anti-SSB (anti-La)

Sjogren syndrome, neonatal lupus, subQ lupus

17

Anti-MPO (myeloperoxidase)

microscopic polyangiitis, eosinophilic granulomatosis w/ polyangiitis (Churg Strauss)

18

Anti-proteinase3

granulomatosis w/ polyangiitis

19

What are some immunosuppressive drugs that are used for treating cancer that could be used as immunosuppressive therapy?

- methotrexate
- cyclophosphamide

20

What are the names of 2 drugs that Nichols mentioned in his word doc that are given to transplant patients to prevent or treat transplant rejections?

- What are their MAO?

Cyclosporine
- tacrolimus

- calcineurin inhibitors

21

What does calcineurin do?

- phosphatase, remove phosphate from the nuclear reg. protein in lymphocytes.

- thus promoting translocation of protein to nucleus to act as TF for lymphocyte activation, TNF, INF-G, IL-2, IL-4.

22

About how much of calcineurin activity does Cyclosporine and Tacroliumus block at therapeutic level?

about half of the activity

23

What is needed for F/U when prescribing Cyclosporin and Tacroliumus?

- regular blood testing

24

What does mycophenolate mofetil do?

- what is it MOA?
- what is it used for?

- inhibits lymphocyte proliferation

- reversible inhibitor of INP DH in purine (guanine) synthesis

- tx autoimmune diseases, particular Lupus and preventing transplant rejection

25

What is azathioprine?

purine analogue
- tx Lupus, R.A, and other autoimmune disases

26

What is hydroxycholorquine?

- anti-malarial antibiotic w/ anti-inflammatory activities.

- tx. Lupus, R.A
- low toxicity

27

Define Systemic Lupus Erythematosus

- prototype multisystem autoimmune disease

28

SLE: epidemiology

Common, 13X more in women, 1/245 black women

29

SLE: pathogenesis

Failure of self-tolerance

30

SLE Gross Pathology:

non-erosive synovitis (inflammation of the synovial membrane) in 90%,
46% pleuritis
25% pericarditis
peritonitis

(basically, all fluid-filled cavities)

- Libman-Sacks endocarditis: nonbacterial endocarditis formed by vegetation.

31

SLE micro path:

acute necrotizing vasculitis of small arteries and arterioles (fibrinoid deposit)
- nephritis in 50%
- cerebritis in 50%

32

SLE symptoms:

- joint pain
- fever
- fatigue
- weight loss
- pleuritic chest pain
- photosensitivity
- nephrotic syndrome
- angina
- alopecia
- myalgias

33

SLE signs:

- skin rash over bridge of nose and check

- edema: 1st in feet
- hematuria
- neuropsychiatric
- oral ulcers
- interarticular skin rashes
- peri-ungual erythema around fingernails
- alopecia

34

SLE dx:

ANA, present in 100%
also present in 15% of normal people

35

SLE tx:

corticosternoids
immunosuppressive meds

36

SLE prognosis:

90% 5 yr survival
80% 10 yr ''''

37

Define Sjogren Syndrome

autoimmune chronic inflammatory diseases of lacrimal glands and salivary glands, causing dry eyes and dry mouth.

- eye: kerato-conjunctivitis sicca
- oral: xerostomia

primary form: limited to eye and mouth --> sicca snydrome

secondary form: associated with autoimmune disorders like R.A, lupus.

38

Sjogren Syndrome Epidemiology:

90% 35-45 y/o women
secondary form more common than primary form

39

Sjogren Syndrome: pathogenesis

T cell mediated immunological attack of self-Ag in ductal epithelial cells.

- or viral infection that has tropism in cells of these glands

40

Sjogren Syndrome Gross Patho:

dry ocular and dry mouth
- enlarged salivary and lacrimal glands

41

Sjogren Syndrome Micro Path:

1) infiltration of CD4 T cells
2) destruction of gland architecture
+/- plasma cells,
+/- germinal centers

Renal: interstitial nephritis rather than glomerulonephritis

42

Sjogren Syndrome Symptoms:

dry eyes, dry mouth

43

Sjogren Syndrome Signs:

dry mucous membrane of eyes and mouth
- conjunctival ulcers
- oral ulcers
- enlarged salivary and lacrimal glands

44

Sjogren Syndrome Dx:

Anti-SSA, SSB.

45

Sjogren Syndrome Tx:

topical therapy
- systemic cholinergic agents
- hydroxychloroquine

46

Sjogren Syndrome prognosis:

notorious for developing lymphoma

47

Systemic Sclerosis definition:

chronic disease characterized by abnormal accumulation of fibrous tissue in skin and other organs

Diffuse: widespread skin, early visceral

limited: skin of fingers, forearms, face, late visceral involvement

within context of CREST syndrome

48

Systemic Sclerosis epidemiology:

10-20 M / year
F:M - 3:1
50-60 y/o
most severe: black women

49

Systemic Sclerosis Pathogenesis

abnormal immune response and vascular damage leads to increase growth factors and fibrosis.

CD4 T cells stimulate fibroblast to produce collagen

Microvascular disease: intimal proliferation, capillary dilation, endothelial injury (increase vWF) + platelet activation --> fibrosis

50

Systemic Sclerosis gross path:

skin
- areas initially edematous, ultimately fibrotic
- begin in face and fingers
- decrease motility leads to masklike face and clawlike hands

GI involvement 90%

51

Systemic Sclerosis micro path:

dense collagen deposition in dermis
- thinning of epidermis
- thickening of capillary/ arterial basal lamina
- vascular changes promote HTN and renal failure, in lung - pulmonary HTN and interstitial fibrosis.

52

Systemic Sclerosis symptoms:

Raynaud's phenomenon
- numbness/tingling/ cyanosis of peripheral skin
- jt pain

53

Systemic Sclerosis sign:

edema of hands and feet, most prominent in the morning

later: thickened, hard / shiny skin -- long bones/ lower legs

54

Systemic Sclerosis Dx:

generalized cutaneous sclerosis, HTN, renal failure, pulmonary HTN and fibrosis.

55

Systemic Sclerosis Tx:

Exercise, splinting, avoid cold

Pharm: antifibrotic
Hemotopoietic stem cell transplantion

56

Systemic Sclerosis Prognosis:

Age, gender adjusted mortality rate increase 5-8 times.

57

Hyperacute Transplant Rejection:

Hypersensitivity Type 2
- vascular injury after attachment (kidney, heart)

- ABO blood type
- cytotoxic Ab against donor organs

-

58

Acute Rejection

w/in 3 months

Cell mediated (most important):
donor macrophage and host T cells

Antibody mediated:
type 2 necrotizing vasculitis with vessel damage and thrombosis

Tx: Cyclosporin A

59

GVH:

Donor lymphocytes produce IL-2, which activates NK cells (effectors of GVH injury)

-