Chapter 139 - Systemic Necrotizing Arteritis Flashcards

1
Q

Matching type

  1. Henoch Schonlein purpura
  2. Kawasaki disease
  3. Giant cell arteritis
  4. Behcet
  5. Polyarteritis nodosa
  6. Churg Strauss Syndrome
  7. Takayasu arteritis
  8. Microscopic polyangiitis

A. Small vessel
B. Medium vessel
C. Large vessel
D. None of the above

A

Small vessel: HSP, Cryoglobulinemia, MPA, CSS, GPA

Medium vessel: Kawasaki, PAN

Large vessel: GCA, TAK, isolated aortitis

No predominant size: Behcet, primary CNS, Relapsing polychondritis, Cogan

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2
Q

Vasculitis becomes palpable when it reaches ___mm in diameter

A

2mm

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3
Q

It is generally recommended biopsy a skin lesion that has been clinically apparent for less than ____ hours

A

48

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4
Q

True or False

Normal liver function tests do NOT rule out infectious hepatitis.

A

True

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5
Q

The most common cutaneous features of polyarteritis nodosa

A

Livedo reticularis/racemosa

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6
Q

All patients with cutaneous vasculitis need to be evaluated for systemic vasculitis.
True or False

A

True

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7
Q

___ is nearly always essential to establish a diagnosis of vasculitis and determine types of vasculitis.

A

Skin biopsy

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8
Q

Mimics of idiopathic vasculitis

A

Infection
Malignancy
Thrombosis
Embolic disease

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9
Q

Essential aspect of management

A

Comprehensive clinical follow up of all patients

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10
Q

Major pathologic process of vasculitis

A

Inflammation of the blood vessels

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11
Q

Skin disease is a frequent manifestation in many forms of vasculitis, esp. ____ arteritis

A

Small and medium vessel arteritis

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12
Q

The most widely used criteria for vasculitis are

A

American College of Rheumatology classification criteria

Chapel Hill Consensus Conference disease definitions

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13
Q

More readily pronounced in medium vessel vasculitis than in small vessel vasculitis

A

Ulceration

Digit ischemia

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14
Q

Classic presentation of small vasculitis

A

Palpable purpura

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15
Q

2 pathways of small vessel vasculitis

A
  1. Immune complex deposition

2. Non-immune complex mediated

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16
Q

ANCA directed against the neutrophil proteins

A
  1. Myeloperoxidase

2. Proteinase -3

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17
Q

Large vessel vasculitides are mediated by T cells activating ___ in walls of large arteries

A

Macrophages

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18
Q

In giant cell arteritis, both __ &; ___ are prominent.

A

IL17 secreting TH17

IFN gamma secreting TH1

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19
Q

Presents with jaw claudication &;shoulder, hip girdle pain

A

Giant cell arteritis

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20
Q

Presents with angina

A

Takayasu arteritis

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21
Q

Similarities of Giant cell arteritis and Takayasu arteritis (5)

A
Amaurosis fugax
Congestive heart failure (aortic valve insufficiency)
Limb claudication
Headaches/ scalp tenderness
Lightheadedness/ Syncope
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22
Q

Gross hematuria due to renal infarction is often seen in

A

Polyarteritis nodosa

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23
Q

Presents with asthma, nasal polyps, &;muscle weakness

A

Churg Strauss syndrome

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24
Q

Presents with proptosis and gingivitis

A

Granulomatous polyangiitis

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25
Q

Similarities of GPA and EGPA (8)

A
Tearing
Ear pain and fullness
Epistaxis, nasal crusting, and discharge, anosmia
Facial pain/ tooth pain
Pulmonary nodules —> hemoptysis
Bronchitis/ large airway collapse —> dyspnea, cough
Myocarditis
Superficial granulomas
26
Q

All small vessel vasculitis presents with (6)

A
Sensorineural hearing loss
Pulmonary emboli
Glomerulo nephritis 
Sensory/motor dysfunction
Purpura
Peripheral edema
27
Q

Presentation of medium to large artery stenosis

A

Digital ischemia/gangrene

28
Q

Single most important component of early evaluation of patient suspected of having vasculitis

A

Full ROS

29
Q

Skin lesions, always purpura, are the most common manifestation of drug-induced vasculitis.
True or False

A

False, usually purpura

30
Q

Normal phyical examination r/o pathology.

True or False

A

False, do not

Eg. Normal BP —> severe glomerulonephritis

31
Q

Helpful measures to screen for large vessel vasculitis: (3)

A

Absent pulses
Asymmetric blood pressure
Bruits

32
Q

Typical features of acute neutrophilic vasculitis

A
Fibrinoid necrosis
Extravasation of RBCs
Extravasation of neutrophils
Leukocytoclasia (nuclear debris)
Immune deposits
33
Q

Extravascular granulomas with geographic necrosis is strongly suggestive of

A

Granulomatosis polyangiitis

34
Q

Predominance of IgA over IgG is diagnostic of IgA vasculitis.
True or False

A

False, suggestive only

35
Q

Most important to order in patient suspected of having vasculitis

A

Tests for renal disease
Urinalysis
Serum creatinine

36
Q

__ vessel vasculitis is expected to produce hematuria, accompanied by RBC casts, and proteinuria; on the other hand, ___ vessel vasculitis is expected to produce isolated hematuria only

A

Small; medium

37
Q

Normal liver function tests cannot rule out infectious hepatitis.
True or False

A

True

38
Q

___ should be ordered on all patients suspected of having vasculitis

A

CBC

39
Q

An elevated eosinophil count greater than ___ cells/ul is found in most untreated patients with Churg Strauss

A

1000

40
Q

Levels of ESR/CRP do not correlate with severity of disease.

True or False

A

True
May have normal values during active vasculitis
May remain in remission despite elevation of markers

41
Q

Approximately __% of patients with MPA

__% of patients with GPA, and __% of patients with EGPA will test positive for ANCA

A

90%
75%
40%

42
Q

ANA is extremely specific for SLE (> 95%).

True or False

A

False, sensitive

43
Q

Only __ % of Sjogren syndrome patients tests positive for RF, anti-Ro, anti-La.

A

80%

44
Q

At least __% of RA tests positive for RF; and at least __% of rheumatoid vasculitis

A

70%

95%

45
Q

In cryoglobulinemic vasculitis, __ levels are usually depleted; and ___ less depleted or even normal

A

C4; C3

46
Q

Low complement helps to raise suspicion for lupus but not specific for vasculitis in SLE. True or False

A

True

47
Q

Drugs of abuse associated with cutaneous vasculits and/or arterial vasospasm

A

Cocaine

Amphetamines

48
Q

Levamisole can lead to characteristic gangrenous lesions as well as positive tests for the ff antibodies

A

Anti-PR3

Anti-MPO

49
Q

Appropriate screening test for vasculitis

A

Chest Xray

50
Q

For patient with vasculitis and pulmonary symptoms, __ is usually indicated

A

CT scan

51
Q

For small vessel vasculitis, a screening __ is indicated for staging

A

CT scan

52
Q

Sinus involvement is extremely common in

A

EGPA

GPA

53
Q

Nasal inflammation is better assessed with a CT scan.

True or False

A

False, by PE

54
Q

Central role in the diagnosis and management of large and medium vessel vasculitis

A

Angiography

55
Q

Nerve conduction testing is recommended for screening asymptomatic patients and to distinguish between compressive and noncompressive neuropathy
True or False

A

False, not recommended

56
Q

__ is a commonly missed manifestation of small vessel vasculitis

A

Hearing loss

57
Q

Baseline __ is recommended for all patients with established diagnosis of ANCA-associated vasculitis

A

Audiogram

58
Q

Medical emergency of rapid onset cardiomyopathy is related to

A

EGPA

59
Q

Angina or myocardial infarction is an expected emergency in

A

Large vessel vasculitis

60
Q

Pulmonary embolus is an expected medical emergency of

A

Small vessel vasculitis