Vasculitic Disorders Flashcards

(44 cards)

1
Q

What can cause vasculitic disorders?

A
  1. type III hypersensitivity (immunocomplex)
  2. type II hypersensitivity (antigen-Ab)
  3. ANCA
  4. Direct invasion by all classes of microbial pathogens
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2
Q

What is ANCA?

A

antibodies against cytoplasmic components of neutrophils

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3
Q

What is cANCA associated with?

A

Wegener granulomatosis

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4
Q

What is pANCA associated with?

A

microcopis polyangiitis

churg strauss syndrome

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5
Q

What is observed with small vessel vasculitis?

A

palpable purpura

acute inflammation

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6
Q

What are examples of small vessel vasculitis?

A

henoch schonelein purpura

microscopic polyangiitis

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7
Q

What is associated with medium sized vasculitis?

A

Thrombosis, infarction, aneurysm of MUSCULAR artery

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8
Q

What are examples of medium sized vasculitis?

A

polyarteritis nodosa

kawasaki disease

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9
Q

What is commonly seen in large vessel vasculitis?

A

loss of pulse/stroke

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10
Q

What are examples of large vessel vasculitis?

A

Takayasu arteritis

giant cell

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11
Q

What is Takayasu arteritis?

A

PULSELESS DISEASE

Granulomatous
LARGE vessel vasculitis
involvies the AORTIC ARCH

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12
Q

What pop is Takayasu arteritis most commonly observed in?

A

young asian women and children

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13
Q

What are the clinical findings and tx for Takayasu arteritis?

A

Absent upper extremity pulse
Discrepancy in bp between arms
visual defects
stroke

tx- corticosteroids

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14
Q

What is a giant cell arteritis?

A

Granulomatous
Large vessel
involves superficial and temporal opthalmic arteries

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15
Q

What population is giant cell arteritis most commonly observed in?

A

adults >50

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16
Q

What are common clinical findings and tx for giant cell arteritis?

A

Temporal HA, jaw claudication
Blindness on ipsilateral side
Polymyalgia rheumatica

Increased ESR

tx. corticosteroids

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17
Q

What is polyarteritis nodosa?

A

Necrotizing
Medium sized vessel
involves renal, coronary, mesenteric arteries

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18
Q

What population is polyarteritis nodosa most commonly seen in?

A

Middle aged men

Asssoc. with hep B

19
Q

What are clinical findings and tx for polyarteritis nodosa?

A

Vessels are at ALL stages of acute and chronic inflammation
Fever
Focal vasculitis> aneurysms
Organ infarction

Angiography and biopsy

tx: corticosteroids, cyclophosphamide

20
Q

What is kawasaki disease?

A

Necrotizing
Medium vessel
coroanry arteries

21
Q

What pop is kawasaki disease seen in?

A

Children G)

Children of asian descent have highest incidence

22
Q

What are clinical findings associated with kawasaki disease?

A

Fever, erythema, edema of hands and feet
strawberry appearing tongue

Abnormal ECG (acute MI)

Tx: ASPIRIN, IV immunoglobulin

23
Q

What is Buerger disease?

A

Medium sized
digital vessel thrombosis
damage to neurovascular compartment

24
Q

What pops does buerger disease occur in?

A

Ashkenazi jews

Men 25-50 who smoke cigarettes

25
What are the common clinical findings and tx for buerger disease?
Resting pain of forefoot and ulcers/gangrene of foot/toes Upper limpb ischemia w/ ulceration and gangrene, raynaud phenomenon tx- smoking cessation, vasodilators
26
What is raynaud disease?
medium sized | digital vessels in fingers and toes, tip of nose and ears
27
What pop is raynaud disease occur in MC?
young women
28
A young woman presents with paroxysmal digital color changes, and ulceration/gangrene of the fingers. What does she have?
Raynaud disease tx- avoid cold temps, Ca channel blockers
29
A man with SLE presents with systemic sclerosis and CREST syndrome as well as digital vasculitis with vessel fibrosis and dystrophic calcification. What does he have?
Raynaud phenomenon
30
What is raynaud phenomenon?
medium sized | digital vessels in fingers, toes, nose, ears
31
Who is affected by raynaud phenomeon?
Adult men and women | secondary to other diseaes (systemic sclerosis, CREST syndrome, SLE)
32
A 41 year old man presents with necrotizing granulomas in the skin, nasopharynx, lungs and kidneys. He also has c-ANCA antibodies. What does he have?
Wegener granulomatosis Equally common in men and women
33
What is wegener granulomatosis?
Necrotizing meidum and small sized vessel involves lung (infarctions) and renal vessels (glomerulonephritis)
34
A child presents with palpable purpura, glomerulonephritis and p-ANCA bodies. The vessels are at the SAME stage of inflammation. What do they have?
Microscopic polyangitis
35
What is microscopic polyangitis?
Small vessel | involves skin, lung, brain, GI tract, kidneys
36
What precipitates Microscopic polyangitis?
Drugs, infections and immune disorders (SLE) Can occur in children and adults tx- corticosteroids and cyclophosphamide
37
A pt with an autoimmune disease presents with allergic rhnitis, asthma, p-ANCA antibodies and eosinophilia. What do he have?
Churg strauss syndrome
38
What is churg strauss syndrome?
small vessel | skin, lung, heart vessels
39
What pop is churg strauss most commonly seen in?
Adults around 51 with autoimmune diseases tx- corticosteroids
40
A 14 year old man presents with palpable purpura of the buttocks and lower extremities, polyarthritis, glomerulonephritis, and abdominal pain and vomiting. He recently had a viral URI. What does he have
Henoch Schonlein purpura tx- corticosteroids
41
What is henoch sconlein purpura?
Type III hypersensitivity small vessel involves skin, GI tract, renal, joint vessels
42
A woman presents with palpable purpura, acral cyanosis of nose and ears and raynaud phenomenon. She also has glomerulonephritis and arthritis. What does she have?
Cryoglobulinemia tx- immunosupressives
43
What is cryoglobulinemia?
(proteins in plasma that gel at cold temperatures particular in areas exposed to cold temp-fingers, nose, etc.) Small vessels Skin, GI, renal vessels More common in females than males
44
A 24 yr old female presents with fever and petechiae that begins on the palms and spreads to the trunk. What does she have?
Infectious vasculitis (RMSF) Small vessels skin vessels tx- IV penicillan G