Lecture 13 Vasculitis Flashcards

1
Q

Vasculitis is ____ of the blood vessel wall which can lead to _____ and _____

A

inflammation;

thrombus, fibrosis

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2
Q

Takayasu stereotypic patient:

Temporal/giant cell stereotypic patient:

A

asian females less than 40;

elderly females

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3
Q

2 large vessel vasculitis:

A

takayasu; temporal/giant cell

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4
Q

Takayasu classically causes _____ thickening and narrowing of the ____ ____ and proximal great vessels

A

granulomatous;

aortic arch

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5
Q

Takayasu is called ____ disease due to a blood pressure difference between the upper and lower extremities

A

pulseless

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6
Q

Treatment for both large vessel vasculitides:

A

high dose corticosteroids

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7
Q

Giant cell arteritis (GCA) typically affects branches of the ____ artery

A

carotid (ie temporal)

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8
Q

in GCA, temporal artery involvement causes ____;

opthalmic artery involvement can cause ____

A

headache, jaw claudication;

blindness/visual problems

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9
Q

GCA is associated with _______ _____, seen as flu-like symptoms, myalgia, and joint pain

A

polymyalgia rheumatica

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10
Q

The 2 large cell vasculitides present with _____ ESR

A

elevated

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11
Q

What kind of inflammation do GCA and takayasu show?

A

granulomatous

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12
Q

What are the 2 medium vessel vasculitides mentioned in the notes?

A

polyarteritis nodosum (PAN), kawasaki disease

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13
Q

What is the most commonly affected organ in PAN, and what are the characteristic findings?

A

Kidney–>hypertension, elevated BUN

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14
Q

If there is ischemia to the GI, symptoms such as ____ and ____ can occur

A

pain, melena

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15
Q

What is characteristically seropositive in PAN?>

A

HBSAg (hep b surface antigen)

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16
Q

treatment for PAN?

A

corticosteroids, cyclophosphamide

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17
Q

Kawasaki disease classically affects ____

A

asian kids less than 4 years

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18
Q

Most important artery that can be affected in kawasaki?

A

coronary artery

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19
Q

‘CRASH and burn’ symptoms associated with kawasaki:

A

Conjunctivitis, Rash, Adenopathy, Strawberry tongue (oral mucositis), Hand and foot rash, and Fever (high)

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20
Q

Treatment for kawasaki?

A

IV immunoglobulin and Aspirin

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21
Q

ANCA positive small vessel vasculitides?

A

microscopic polyangiitis, granulomatosis with polyangititis (wegener’s), eiosinophillic granulomatosis with polyangitis (churg strauss)

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22
Q

what immunoassay enzyme is associated with c-ANCA? what is the characteristic staining?

A

proteinase 3 (PR3); cytoplasmic

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23
Q

what immunoassay enzyme is associated with p-ANCA?; what is the characteristic staining?

A

myeloperoxidase (MPO); perinuclear

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24
Q

Memory hook for Wegener’s/granulomatosis with polyangiitis (GPA):
characterisitc areas affected:

A

“C” disease (from pathoma)

nasopharynx, lungs, kidneys

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25
Q

ANCA associated with GPA?

A

c-anca/PR3

remember C disease

26
Q

treatment for GPA?

A

Corticosteroids and cyclophosphamide

remember C disease

27
Q

Nasal involvement with GPA presents as _____

A

saddle nose

28
Q

Microscopic polyangiitis (MPA) classically affects the ____ and _____

A

lungs, kidneys

(similar to GPA minus nasopharynx involvement

29
Q

what ANCA is associated with MPA?

A

p-anca/myeloperoxidase

30
Q

What kind of skin/renal infection does MPA cause?

A

“pauci-immune”

31
Q

Cardinal features of eosinophillic granulomatosis with polyangiits (CS for churg strauss)? ____, ____, and lung disease

A

asthma, sinusitis

32
Q

Churg strauss is associated with _____neuritis

A

mono (ie wrist drop)

33
Q

3 elevated labs with churg strauss:

A

eiosinophillia, P-ANCA (MPO-ANCA), IgE

34
Q

What can Churg strauss can be a complication of treatment with?

A

montelukast (singulair) or zafirlukast (accolate)

35
Q

What lung symptom does antiglomerular basement membrane disease (goodpasture’s) often present with?

A

pulmonary hemorrhage

36
Q

anti ______ ____ _____ antibodies should be demonstrated in diagnosis of goodpasture’s

A

glomerular basement membrane

37
Q

_____ vasculitis is due to precipitation of blood proteins at temperatures lower than 37 degree C

A

cryoglobulinemic

38
Q

Type 1 cryoglubulins are ______ while type 2 and 3 are _____

A

monoclonal; polyclonal

39
Q

Type 1 cryoglobulinemia can cause _____ of blood in the brain, leading to neurologic symptoms

A

hyperviscosity

40
Q

IgA associated vasculitis (Henoch Scholein Purpura) is characterized by what skin condition, and where is it located?

A

palpable purpura–lower extremities ie buttocks

41
Q

What is Henoch Scholein Purprua secondary to?

A

IgA immune complex deposition

42
Q

What is another name for palpable purpura?

A

leukocytic vasculitis

43
Q

______ _____ vasculitis usually presents as uticaria that persists for up to 72 hours (more than normal hives)

A

hypocomplementic uticarial

44
Q

What is seen in hypocomplementemic uticaria that is not seen in regular uticaria?

A

pain

45
Q

Classic lab findings of hypocomplementmic uticaria?

A

decreased complements, positive ANA

46
Q

pathology of hypocomplementimic uticaria?

A

leukocytic vasculitis

47
Q

Classic presentation of behcet’s disease? ulceration of the ____ and ____

A

mouth and genitals o_o

48
Q

the ____ test is when pustule like lesions appear 48 hours after a prick with a needle. it is positive in ____

A

pathergy; behcet’s disease

49
Q

behcet’s is common along the _____ ____

A

silk road (ie east asia to mediterranean)

50
Q

Classic symptoms of cogan’s syndrome: ____ ____ of the eyes and ______ dysfunction

A

interstitial keratitis; vestibuloauditory

51
Q

what blood vessel is commonly associated with cogan’s vasculitis?

A

aorta

52
Q

what is the classic appearance of drug associated immune complex vasculitis?

A

cutaneous leukocytoplastic angiitis (palpable purpura)

53
Q

what is the name of a single organ vasculitis that presents as seizures, stroke, TIA, or other neurological defects?

A

primary CNS vasculitis

54
Q

Characteristic CSF findings of primary CNS vasculitis:
_____ pleocytosis with oligoclonal bands;
elevated _____

A

lymphoctyic

protein

55
Q

What is the angiographic pattern seen in CNS vasculitis?

A

alternating stenosis and dilations (beading pattern)

56
Q

what is commonly mistaken for primary CNS vasculitis?

A

reversible cerebral vasoconstriction syndrome (RCVS)

57
Q

what is the presenting symptom most commonly seen in RCVS?

A

hyperacute thunderclap headache

58
Q

‘treatment’ of RCVS?

A

typically observation

59
Q

What disease in usually seen in heavy male smokers

A

thromboangiitis obliterans (buerger’s disease)

60
Q

what is the most common symptom of buerger’s disease?

A

digital finger ischemia (ie raynauds, gangrene, pain)

61
Q

Most important aspect of treatment for buerger disease?

A

STOP SMOKING