Immunopathology III Vasculitis- Friedlander Flashcards by Jes Testa

definition of vasculitis?

inflammation of blood vessel wall

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vasculitis of large vessel?

polyarteritis nodosa

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what layers of vessel involved with PAN?

all 3 layers

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what etiologies are associated with PAN?

hep B, C, and cryoglobulins

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how does PAN present clinically?

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

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How does PAN present grossly?

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

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what is vasculitis of a small vessel?

Microscoptic polyangitis

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what vessels are involved in MSPA?

small veins and a. thru 3 layers

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what test is positive with MSPA?

p-ANCA; perinuclear pattern

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Clinical SXS of MSPA?

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

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what is Granulomatosis with polyangitis?

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

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what test is positive with Wegner’s?

c-ANCA : Abs against proteinase 3 (neutrophils)

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What does Wegner’s look like histologically?

Granulomas, necrosis (coagulation), RPGN III

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what does wegner’s look like grossly?

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

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what is the classical presentation of Kawasaki’s Disease?

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

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what is the treatment for Kawasaki’s?

Aspirin

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what is the most common childhood vasculitis?

Henoch-Schonlein purpura

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what is the classic tetrad for Henoch-Schonlein Purpura?

skin- palpable purport on buttocks/legs
arthralgias
GI- abdominal pain, melena
IgA Nephropathy

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what is Churg-Strauss?

granulomatous necrotizing vasculitis with eosinophilia

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what organs are involved in Churg-Strauss?

multiple: heart and lungs

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what test is positive in Churg-Strauss?

p-ANCA+

What does C-S present as?

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

What does Behcet’s present with?

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

what vessels are affected by Bechet’s?

a. and v. of ALL sizes

why is the pathophysiology of Bechet's?

T-cells against HSP-60

How is Bechet's diagnosed?

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

what is amyloid?

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

what does Classic amyloid stain look like?

congo red stain show apple-green birefringence under polarized light

what organ is affected most by amyloidosis?

kidney

cryoglobulinemia is what?

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

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

sago or lardaceous

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

carpal tunnel syndrome

how did amyloidosis previously present?

post-proctoscopy palpebral purpura

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

high neutrophil count because no diapedesis

what is chediak-higashi dz a defect in?

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

what is chronic granulomatous dz a defect in?

oxygen-dependent bacterial killing mechanisms in macrophages

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

lupus; alternate pathway works fine and handles bacteria

deficiency in C3 causes?

serious problems with bacteria and with immune complex membranoproliferative glomerulonephritis

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

neisseria

C1 (esterase) inhibitor produces?

hereditary angioedema

what is X-linked SCID usually due to?

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

what does X linked agammaglobulinemia present as?

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

pts with DiGeorge's syndrome lack?

parathyroids and thymus and have midline cardiac defects

what is hyper-IgM?

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

Isolated IgA deficiency occurs when?

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

sex-linked lymphoproliferative syndrome

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

what does Wiskott-Aldrich present with?

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

what does ataxia-telangiectasia present with?

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

Hyper IgE syndrome?

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

what syndrome did bubble boy have?

SCID