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Mechanisms of Disease > Immunopathology III Vasculitis- Friedlander > Flashcards

Immunopathology III Vasculitis- Friedlander Flashcards

1
Q

definition of vasculitis?

A

inflammation of blood vessel wall

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2
Q

vasculitis of large vessel?

A

polyarteritis nodosa

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3
Q

what layers of vessel involved with PAN?

A

all 3 layers

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4
Q

what etiologies are associated with PAN?

A

hep B, C, and cryoglobulins

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5
Q

how does PAN present clinically?

A

symptoms related to organ involved; kidneys= HTN, no hematuria

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6
Q

How does PAN present grossly?

A

MRI see aneurysms, starts as small tissue infarcts, black spots/holes in skin

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7
Q

what is vasculitis of a small vessel?

A

Microscoptic polyangitis

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8
Q

what vessels are involved in MSPA?

A

small veins and a. thru 3 layers

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9
Q

what test is positive with MSPA?

A

p-ANCA; perinuclear pattern

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10
Q

Clinical SXS of MSPA?

A

hematuria!!: RPGN III**, MI, Stroke, Hemoptysis & infiltrates, gangrene, PNS

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11
Q

what is Granulomatosis with polyangitis?

A

Wegner’s Granulomatosis; necrotizing vasculitis involving nasopharyns, lungs, and kidneys

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12
Q

what test is positive with Wegner’s?

A

c-ANCA : Abs against proteinase 3 (neutrophils)

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13
Q

What does Wegner’s look like histologically?

A

Granulomas, necrosis (coagulation), RPGN III

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14
Q

what does wegner’s look like grossly?

A

saddle nose, hole in palate, red gingivitis on gums, tongue & foot ulcers, Lung infiltrates

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15
Q

what is the classical presentation of Kawasaki’s Disease?

A

Kids, fever, Japanese ancestry; presents like viral illness: fever, conjunctivitis, rash, strawberry tongue, red palms and soles, enlarged cervical LN

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16
Q

what is the treatment for Kawasaki’s?

A

Aspirin

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17
Q

what is the most common childhood vasculitis?

A

Henoch-Schonlein purpura

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18
Q

what is the classic tetrad for Henoch-Schonlein Purpura?

A
  1. skin- palpable purport on buttocks/legs
  2. arthralgias
  3. GI- abdominal pain, melena
  4. IgA Nephropathy
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19
Q

what is Churg-Strauss?

A

granulomatous necrotizing vasculitis with eosinophilia

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20
Q

what organs are involved in Churg-Strauss?

A

multiple: heart and lungs

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21
Q

what test is positive in Churg-Strauss?

A

p-ANCA+

22
Q

What does C-S present as?

A

asthma + eosinophils; looks like polyarteritis or Wegener’s but with eosinophils

23
Q

What does Behcet’s present with?

A

ALWAYS mouth ulcers and maybe genitals, inflammation of iris and ciliary body, neuro symptoms, amyloidosis, thrombosis

24
Q

what vessels are affected by Bechet’s?

A

a. and v. of ALL sizes

25
Q

why is the pathophysiology of Bechet’s?

A

T-cells against HSP-60

26
Q

How is Bechet’s diagnosed?

A

with a pathergy test: sterile pin into skin: canker sore in 48hrs

27
Q

what is amyloid?

A

“beta pleated anything” abnormal aggregation of proteins into beta-pleated sheets: PINK!

28
Q

what does Classic amyloid stain look like?

A

congo red stain show apple-green birefringence under polarized light

29
Q

what organ is affected most by amyloidosis?

A

kidney

30
Q

cryoglobulinemia is what?

A

marginally soluble protein precipitates in the cooler places and/or where blood is concentrated, can cause vasculitis

31
Q

what are the 2 ways that amyloid of the spleen present as?

A

sago or lardaceous

32
Q

what syndrome (think upper extremities) is often associated with amyloidosis?

A

carpal tunnel syndrome

33
Q

how did amyloidosis previously present?

A

post-proctoscopy palpebral purpura

34
Q

in patients without leukocyte adhesion molecules what will blood look like?

A

high neutrophil count because no diapedesis

35
Q

what is chediak-higashi dz a defect in?

A

phagolysosome function; protein trafficking problem, also interferes with melanosome formation and causes neuropathy

36
Q

what is chronic granulomatous dz a defect in?

A

oxygen-dependent bacterial killing mechanisms in macrophages

37
Q

deficiencies in C2, C4, and especially C1q mimic what dz?

A

lupus; alternate pathway works fine and handles bacteria

38
Q

deficiency in C3 causes?

A

serious problems with bacteria and with immune complex membranoproliferative glomerulonephritis

39
Q

deficiency in C5, 6, 7, 8, or 9 causes issues with what bacteria?

A

neisseria

40
Q

C1 (esterase) inhibitor produces?

A

hereditary angioedema

41
Q

what is X-linked SCID usually due to?

A

mutation in common g-chain of the interleukin receptors (IL2RG)

42
Q

what does X linked agammaglobulinemia present as?

A

trouble with bacteria, giardia, and enteroviruses at the age of 6 months after mom’s Abs are gone

43
Q

pts with DiGeorge’s syndrome lack?

A

parathyroids and thymus and have midline cardiac defects

44
Q

what is hyper-IgM?

A

pts unable to switch-over to making IgG, IgA, IgE; gene encoding CD 40 on X chromosome is mutated

45
Q

Isolated IgA deficiency occurs when?

A

2nd blood transfusion, anaphylaxis, allergic to donor’s IgA

46
Q

sex-linked lymphoproliferative syndrome

A

portion of signal transduction protein defective, poor response to viruses causes deadly lymphoma-like overgrowth of lymphocytes, or destroy liver and/or marrow

47
Q

what does Wiskott-Aldrich present with?

A

tiny platelets, eczema, deficient T-cells, X-linked, WASp locus

48
Q

what does ataxia-telangiectasia present with?

A

T-cell counts and/or low antibody levels sometimes; autoimmunity and cancer are common

49
Q

Hyper IgE syndrome?

A

too little antibodies with faulty neutrophil movement; staph is most troublesome

50
Q

what syndrome did bubble boy have?

A

SCID

Mechanisms of Disease (8 decks)

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