Vasculitic Disorders Flashcards

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
Q

What are the common clinical findings and tx for buerger disease?

A

Resting pain of forefoot and ulcers/gangrene of foot/toes
Upper limpb ischemia w/ ulceration and gangrene, raynaud phenomenon

tx- smoking cessation, vasodilators

26
Q

What is raynaud disease?

A

medium sized

digital vessels in fingers and toes, tip of nose and ears

27
Q

What pop is raynaud disease occur in MC?

A

young women

28
Q

A young woman presents with paroxysmal digital color changes, and ulceration/gangrene of the fingers. What does she have?

A

Raynaud disease

tx- avoid cold temps, Ca channel blockers

29
Q

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?

A

Raynaud phenomenon

30
Q

What is raynaud phenomenon?

A

medium sized

digital vessels in fingers, toes, nose, ears

31
Q

Who is affected by raynaud phenomeon?

A

Adult men and women

secondary to other diseaes (systemic sclerosis, CREST syndrome, SLE)

32
Q

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?

A

Wegener granulomatosis

Equally common in men and women

33
Q

What is wegener granulomatosis?

A

Necrotizing
meidum and small sized vessel
involves lung (infarctions) and renal vessels (glomerulonephritis)

34
Q

A child presents with palpable purpura, glomerulonephritis and p-ANCA bodies. The vessels are at the SAME stage of inflammation. What do they have?

A

Microscopic polyangitis

35
Q

What is microscopic polyangitis?

A

Small vessel

involves skin, lung, brain, GI tract, kidneys

36
Q

What precipitates Microscopic polyangitis?

A

Drugs, infections and immune disorders (SLE)

Can occur in children and adults

tx- corticosteroids and cyclophosphamide

37
Q

A pt with an autoimmune disease presents with allergic rhnitis, asthma, p-ANCA antibodies and eosinophilia. What do he have?

A

Churg strauss syndrome

38
Q

What is churg strauss syndrome?

A

small vessel

skin, lung, heart vessels

39
Q

What pop is churg strauss most commonly seen in?

A

Adults around 51 with autoimmune diseases

tx- corticosteroids

40
Q

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

A

Henoch Schonlein purpura

tx- corticosteroids

41
Q

What is henoch sconlein purpura?

A

Type III hypersensitivity

small vessel
involves skin, GI tract, renal, joint vessels

42
Q

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?

A

Cryoglobulinemia

tx- immunosupressives

43
Q

What is cryoglobulinemia?

A

(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
Q

A 24 yr old female presents with fever and petechiae that begins on the palms and spreads to the trunk. What does she have?

A

Infectious vasculitis (RMSF)

Small vessels
skin vessels

tx- IV penicillan G