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Fitz 9th > 139 - Systemic Necrotizing Arteritis > Flashcards

139 - Systemic Necrotizing Arteritis Flashcards

1
Q

Predominantly small vessel vasculitis

A

Immune complex mediated:

  • Cryoglobulinemia
  • HSP

Limited skin

ANCA-associated:

  • MPA
  • CSS
  • GPA
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2
Q

Predominantly medium vessel

A

Kawasaki

PAN

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3
Q

Predominantly large vessel

A

GCA
TAK
Isolated aortitis

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4
Q

No predominant size

A

Behcet
Primary CNS
Relapsing polychondritis
Cogan

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5
Q

Takayasu arteritis is substantially more common in

A

Women

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6
Q

Kawasaki disease is almost exclusively a disease of

A

Young children

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7
Q

Giant cell arteritis is limited to

A

Older adults

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8
Q

Granulomatosis with polyangitis (Wegener) mostly occurs in

A

Whites

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9
Q

Behcet disease is markedly more common in countries in the

A

Eastern Mediterranean
Japan
Korea

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10
Q

Infection-related vasculitis: _____ in many cases of polyarteritis nodosa

A

Hepatitis B virus

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11
Q

Infection-related vasculitis: _____ in most cases of cryoglobulinemic vasculitis

A

Hepatitis C virus

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12
Q

Small-vessel vasculitis follow one of 2 pathways:

A
  1. Immune complex deposition

2. Non-immune complex mediated pathology that likely involves ANCA

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13
Q

ANCA are directed against the neutrophil proteins

A

Myeloperoxidase (MPO)

Proteinase-3 (PR3)

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14
Q

Large-vessel vasculitis are thought to be medicated by

A

T cells activating macrophages

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15
Q

HLA locus in GCA

A

Class II

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16
Q

HLA locus in Takayasu arteritis

A

Class I

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17
Q

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

A

Full review of systems with assessment of overall severity of illness

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18
Q

The diseases that cause cutaneous vasculitis can affect all organ systems, and in most cases that involvement will cause symptoms, _____ being a prominent exception

A

Renal disease

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19
Q

For drug-induced vasculitis, it is useful to ask about prescription, nonprescription, and “alternative” or herbal medication use in the prior

A

6 to 12 months

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20
Q

It is generally recommended to biopsy a skin lesion that has been clinically apparent for

A

Less than 48 hours

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21
Q

Histopathology: extravascular granulomas with geographic necrosis

A

Granulomatosis with polyangitis

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22
Q

Histopathology: eosinophil-rich extravascular granulomas

A

Eosinophilic granulomatosis with polyangitis

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23
Q

Y/N: Kidney or lung biopsies are less likely than skin biopsies to show pathology diagnostic of a particular disease

A

No - more likely

24
Q

Tests for _____ are the most important to order in evaluating a patient suspected of having vasculitis

A

Renal disease

Common in many vasculitides and is rarely accompanied by signs or symptoms until end-stage renal failure occurs

25
Q

An elevated absolute eosinophil is found in most untreated patients with eosinophilic granulomatosis with polyangitis (Churg-Strauss), and a count greater than _____ helps differentiated this disease from asthma and atopy

A

1000 cells/microL

26
Q

Y/N: The levels of ESR and CRP do not correlate well with stage or severity of disease

A

Yes

27
Q

Autoimmune serologies: ANCA-associated vasculitis and lupus can present with vasculitis of the skin, but _____ does not

A

Anti-glomerular basement membrane (GBM) disease

28
Q

90% of patients with _____ will test positive for ANCA

A

Microscopic polyangiitis

29
Q

75% of patients with _____ will test positive for ANCA

A

Granulomatosis with polyangiitis

30
Q

40% of patients with _____ will test positive for ANCA

A

Eosinophilic granulomatosis with polyangiitis

31
Q

Pauci-immune glomerulonephritis, pulmonary hemorrhage, peripheral and cranial neuropathy, musculoskeletal, constitutional symptoms
Most patients are positive for ANCA, usually with specificity for antibodies to myeloperoxidase

A

Microscopic polyangiitis

32
Q

Encompasses all features of microscopic polyangiitis but also many additional manifestations caused by necrotizing granulomatous inflammation
Chronic inflammation of the upper airway ( nasal cavity, sinuses, auditory tube, middle ear), cavitary pulmonary nodules, orbital pseudotumor, subglottic stenosis, vasculitis of the eye
Neutrophilic and granulomatous dermatitis with papules (particularly on the extensor surfaces of the elbows), subctaneous nodules and ulcers
Majority ANCA type is c-ANCA/anti-PR3

A

Granulomatosis with polyangiitis

33
Q

Unique features: history of asthma (often severe or poorly controlled), blood eosinophilia
Nasal polyps, constitutional symptoms, rashes, all typical of atopy
Pulmonary infiltrates on chest imaging (eosinophilic pneumonia)

A

Eosinophilic granulomatosis with polyangiitis

34
Q

Most common presentation of severe vasculitis in eosinophilic granulomatosis with polyangiitis

A

Acute peripheral neuropathy with involvement of the heart, GI tract, brain, eyes

35
Q

Cutaneous disease is more common in (MPA, GPA, EGPA)

A

Eosinophilic granulomatosis with polyangiitis

36
Q

Histologic term defined by the absence of evidence of systemic disease

A

Cutaneous leukocytoclastic angiitis

37
Q

Cutaneous leukocytoclastic angiitis: _____% of cases follow a wide range of infections

A

20

38
Q

Cutaneous leukocytoclastic angiitis: _____% of cases are associated with drug exposure

A

20

39
Q

Skin disease, myalgia, hypertension (from renal artery involvement), abdominal pain, neuropathy and/or testicular pain

A

“Classic” polyarteritis nodosa

40
Q

Most common cutaneous features of polyarteritis nodosa

A

Livedo reticularis/racemosa

41
Q

Disease of adults older than 50 years
Mostly a disease of people of Northern European ancestry
Cranial arteritis is a common feature, polymyalgia rheumatica, fever, malaise, weight loss

A

Giant cell arteritis

42
Q

Diagnosis of GCA is usually confirmed by

A

Temporal artery biopsy

43
Q

Only cutaneous manifestation of GCA apparent on examination

Present in 30% to 40% of cases

A

Palpable nodularity of the temporal artery

44
Q

Rare form of vasculitis involving the aorta and its major branches
Many patients are diagnosed as young adults, 90% are female

A

Takayasu arteritis

45
Q

Typical presentation of Takayasu arteritis

A

Limb claudication

46
Q

Diagnosis of Takayasu arteritis is made by

A

Angiography

47
Q

Mortality in the first 3 to 6 months of severe vasculitis affecting vital organs is around

A

10%

48
Q

Other than advanced age, the predictors of higher risk of mortality reflect prior organ damage: _____ damage in the case of granulomatosis with polyangiits and microscopic polyangiitis, and _____ damage in the case of eosinophilic granulomatosis with polyangiitis

A

Kidney

Cardiac

49
Q

For vasculitides expected to have extended courses and/or to include severe manifestations, the general approach is to plan for 2 phases of treatment:

A

Remission induction

Remission maintenance

50
Q

Mainstay of therapy for vasculitis

A

Glucocorticoids

51
Q

Proven effective for ANCA-associated vasculitides and, to a lesser extent, other forms of vasculitis

A

Cyclophosphamide

52
Q

Serious toxicities of cyclophosphamide which are related to total cumulative dose

A

Female and male infertility

Bladder cancer

53
Q

Apremilast has now been demonstrated to have efficacy in the treatment of

A

Mucocutaneous manifestations of Behcet disease

54
Q

Biologic demonstrated to be as effective as cyclophosphamide for induction of remission of AAV

A

Rituximab

55
Q

Monoclonal antibody to anti-interleukin 5

Demonstrated to have efficacy in the treatment of eosinophilic granulomatosis with polyangiitis

A

Mepolizumab

56
Q

Monoclonal antibody to interleukin-6

Demonstrated efficacy for the treatment of giant cell arteritis

A

Tocilizumab

57
Q

CTLA-4 immunoglobulin

Promising data on its use for giant cell arteritis

A

Abatacept

Fitz 9th (108 decks)

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