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1 Path Unit > 16 Vasculitis > Flashcards

16 Vasculitis Flashcards

1
Q

Giant Cell (Temporal) arteritis affects what group most commonly?

A

Over 50. Especially Northern Europeans.

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2
Q

Giant Cell (Temporal) arteritis affects which arteries? (4)

A

temporal, vertebral, ophthalmic, aorta[group as mostly “arteries of the head”]

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3
Q

T/F temporal arteritis can cause permanent blindness?

A

T. (ophthalmic artery)

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4
Q

Suspected etiology/pathogenesis temporal arteritis?

A

Unknown. Suspected: immune rxn against arterial wall protein, probably T-cell mediated (granulomatous)

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5
Q

Symptoms of temporal arteritis?

A

fever, fatigue, weight loss, abrupt diplopia or vision loss[note most are non-specific]

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6
Q

Treatment temporal arteritis?

A

high-does anti-inflammatories (steroids)

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7
Q

Risk factors: Takayasu arteritis? (2)

A

-female under 40 (8:1, f:m)-HLA -A24, B52, DR2

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8
Q

Incidence: Takayasu arteritis? (2)

A

-US: 1/1000-Asia: 6/1000

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9
Q

Giant Cell (Takayasu) arteritis: affects what body parts? (3) How?

A

-Ascending aorta “Pulseless disease”-Can also involve pulmonary & renal arteries-Fibrosis of artery wall ->stenosis-> occlusion->dilation-> aneurysm

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10
Q

Giant cell (Takaysu) arteritis: signs early & late phases?

A

-Early: fever, arthalgias, weight loss, “prepulseless”-Late: “Pulseless”

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11
Q

Type 1 Takayasu Arteritis is?

A

branchiocephalic trunk, carotid arteries & subclavian

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12
Q

Type 2 Takayasu Arteritis is?

A

combined Type 1 & Type 3

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13
Q

Type 3 Takayasu Arteritis is?

A

“atypical coarctation type” involves thoracic & abdominal aorta (most common!!)

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14
Q

Type 4 Takayasu Arteritis is?

A

“Dilated type”, dilation of aorta & its major branches

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15
Q

Takayasu arteritis: treatment? (2)

A

-steroids-angioplasty (after acute phase)

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16
Q

Thromboangitis obliterans (Buerger’s disease): Risk factors? (3)

A

-Japan or Near East descent-Smoking-HLA-A9, HLA-B5

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17
Q

Thromboangitis obliterans: etiology, pathogenesis? (3)

A

-Endothelial damage-Hypersensitivity to tobacco extract-Result: acute & chronic vasculitis of medium & small arteries, esp radial & tibial. -> Ulceration of fingers & toes

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18
Q

Thromboangitis obliterans: treatment?

A

Quit smoking!

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19
Q

Wegener’s granulomatosis (Granulomatosis with polyangitis): risk factors?

A

-Any age, sex, race, but mostly whites w/ slightly more males-Age 41 median onset

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20
Q

Wegener’s granulomatosis (Granulomatosis with polyangitis): etiology, pathogenesis? (4)

A
  • Unknown. Suspected: Hypersensitivity rxn initially
  • Necrotizing granulomatous vasculitis
  • Focal segmental glomerular nephritis
  • Necrotizing vasculitis of median & small vessels
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21
Q

Kawasaki’s/Mucocutaneous Lymph Node Syndrome: risk factors? (2)

A
  • Under 4 yrs

- USA or Japan [#1 ACQUIRED hearts disease in these countries]

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22
Q

Kawasaki’s/Mucocutaneous Lymph Node Syndrome: pathogenesis?

A

-anti-endothelial, anti-smooth muscle Ab’s, and T-cell attack ->coronary artery vasculitis-> Possible MI, rupture, ectasia, or thrombosis.

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23
Q

Kawasaki’s/Mucocutaneous Lymph Node Syndrome: etiology

A

possibly viral infection

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24
Q

Kawasaki’s/Mucocutaneous Lymph Node Syndrome: symptoms?

A

fever, rash, swollen hands/feet w/ desquamation, red eyes, swollen neck lymph nodes, strawberry tongue/mouth/throat

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25
Q

Kawasaki’s/Mucocutaneous Lymph Node Syndrome: treatment?

A

aspirin, IVIG

26
Q

Polyarteritis Nodosa: What does it affect? (3)

A
  • small-medium vessels
  • May involve any organ. Commonly skin, joints, peripheral nerves, GI, kidney
  • Often with Hepatitis B
27
Q

Polyarteritis Nodosa: pathogenesis? (4)

A
  • panmural necrotizing arteritis with fibrinoid necrosis! esp. at branch pt (either circumferential or segmental)
  • mixed cell infiltrate
  • aneurysmal dilatation
  • heal w/ fibrosis
28
Q

Polyarteritis Nodosa: diagnosis? (2)

A
  • angiography or histology

- ANCA 80% positive, equally distributed c and p forms

29
Q

Polyarteritis Nodosa: treatment/prognosis?

A
  • glucocorticoids

- half die in 5 yrs, most in first year

30
Q

which CAN have granulomas: polyarteritis nodosa or microscopic polyangitis?

A

Poly nodosa

31
Q

which has medium and small vessels: polyarteritis nodosa or microscopic polyangitis?

A

poly nodosa (MICROSCOPIC polyangitis has only SMALL)

32
Q

Which has glomerulonephritis: polyarteritis nodosa or microscopic polyangitis?

A

microscopic polyangitis

33
Q

Which has pulmonary hemorrhage: polyarteritis nodosa or microscopic polyangitis?

A

microscopic polyangitis

34
Q

Which has relapses frequently? polyarteritis nodosa or microscopic polyangitis?

A

microscopic polyangitis

35
Q

Which has abnormal angiography: polyarteritis nodosa or microscopic polyangitis?

A

poly nodosa

36
Q

Which has renal vasculitis w/ renal hypertension & infarcts: polyarteritis nodosa or microscopic polyangitis?

A

poly nodosa

[note: micro polyangitis had glomerulonephritis!]

37
Q

Wegener’s granulomatosis (Granulomatosis with polyangitis): manifestations? (3)

A
  • sinusitis, nasal obstruction, otitis media, hearing loss, skin rash, fever, arthralgia, weight loss
  • lung involvement: 45% at presentation ->85% in all. Pulm nodules, hemoptosis, pleuritis
  • 80% glomerulonephritis
38
Q

Wegener’s granulomatosis (Granulomatosis with polyangitis): diagnosis? (5)

A
  • Lung biopsy
  • Vessels w/ fibrinoid necrosis + PMN’s
  • renal biopsy
  • gingival biopsy
  • ANCA: 90% c and 10% p
39
Q

Wegener’s granulomatosis (Granulomatosis with polyangitis): treatment & prognosis? (2)

A
  • cyclophosphamide (chemotherapy)

- remission partial in 90%, full in 75%, 1/2 relapse in 3 yrs

40
Q

Eosinophilic granulomatosis with polyangiitis a.k.a. allergic angitis a.k.a. granulomatosis a.k.a. Churg Strauss: risk factors? (1)

A

-25-69 yrs, median 38-50

41
Q

Eosinophilic granulomatosis with polyangiitis a.k.a. allergic angitis a.k.a. granulomatosis a.k.a. Churg Strauss: pathogenesis? (3)

A
  • vascular & extravascular granulomatous inflammation
  • allergic rhinitis, severe asthma, multiple organs
  • hypereosinophilia
42
Q

Eosinophilic granulomatosis with polyangiitis a.k.a. allergic angitis a.k.a. granulomatosis a.k.a. Churg Strauss: clinical course? (3)

A

Phases:

  • Prodromal: allergic respiratory disease
  • Eosinophilic: peripheral blood & tissue eosinophilia
  • Vasculitic
43
Q

Eosinophilic granulomatosis with polyangiitis a.k.a. allergic angitis a.k.a. granulomatosis a.k.a. Churg Strauss: diagnosis?

A

-ANCA 70-80% positive, usually P & anti-MPO

44
Q

Behcet’s Disease: risk factors?

A

-middle east, japan, china “silk route”

45
Q

Behcet’s Disease: Manifestations? (6)

A
  • All artery types
  • aphthous ulcers in mouth
  • pustular lesions on skin & scarring erythema nodosum
  • scrotal, vulvar ulcers
  • uveitis
  • lung aneurysms
46
Q

Behcet’s Disease: diagnosis?

A

pathergy test (sterile pin evokes allergic rxn!)

47
Q

Behcet’s Disease: treatment? (2)

A

immunosuppression, thalidomide

48
Q

Primary CNS Vasculitis: what’s the only type that gets better over time?

A

BACNS (benign angiopathy of the CNS)

49
Q

Primary CNS Vasculitis: risk factors?

A

4:3 female to male. BACNS typically is in young women.

50
Q

Primary CNS Vasculitis: symptoms? (8)

A
  • headache
  • organic brain syndrome
  • multifocal neurologic deficits
  • seizures
  • strokes
  • confusion
  • memory loss
  • altered consciousness
51
Q

Primary CNS Vasculitis: etiology?

A

maybe coffee, nicotine, or contraceptives, unknown really.

52
Q

Primary CNS Vasculitis: diagnosis?

A
  • angiography or MRI (still not certain)

- Brain and meningeal biopsy (25% false negatives still)

53
Q

Primary CNS Vasculitis: treatment?

A

massive immunosuppression. Usually fatal within 45 days.

54
Q

BCANS (benign angiopathy of the CNS) subset of CNS vasculitis: treatment?

A

Ca channel blockers, short course steroids, get better!

55
Q

Raynaud’s phenomena classic risk factors?

A

woman in cold climate w/ emotional stress, age 20-40, with a cigarette in one hand, a bottle of alcohol in the other hand, and munching on an estrogen replacement pill in her mouth.

56
Q

Raynaud’s phenomena color change?

A

white, blue, red

[no blood, vessels dilate to keep blood, flow returns]

57
Q

Raynaud’s phenomena has what causes? (2)

A
  • Primary, less severe & bilateral

- Secondary: not bilateral, associated w/ connective tissue disorder

58
Q

Raynaud’s phenomena treatment?

A

eliminate the risk factors, and give sympathetic nerve blockers

59
Q

Raynaud Syndrome etiology & pathogenesis?

A

cryoglobulins occlude vessels @ low temp ->purpuric lesions

60
Q

Raynaud Syndrome risk factors? (3)

A
  • collagen vascular diseases (SLE, R.A., Scleroderma)
  • infectious disease
  • hematologic disorders
61
Q

Raynaud Syndrome treatment?

A

keep warm, treat underlying disease

1 Path Unit (16 decks)

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