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Clin Med IV - Ortho > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

When might you suspect vasculitis?

A

Pt with constitutional sx’s and single/multi-organ dysfunction

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2
Q

Vasculitis is associated with what autoimmune disorder?

A

SLE

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3
Q

Clinical presentation of vasculitis

A
  • Constitutional sx’s
  • Skin → palpable purpura, livedo reticularis
  • Nervous system → monoeurieitis multiplex, reduced visual acuity, stroke
  • Eye → anterior/posterior uveitis, scleritis
  • CV → HTN, MI
  • Respiratory → epistaxis, hemoptysis, pulmonary infiltrates
  • Kidney → glomerulonephritis, HTN
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4
Q

________ + ________ should raise concern for vasculitis

A

Lung hemorrhage
Renal insufficiency
(Pulmonary-renal syndrome)

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5
Q

Standard imaging for screening for large-vessel vasculitis

A

CT, MRI but no angiographic abnormalities are pathognomonic for vasculitis → need clinical support + data

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6
Q

Name the 2 types of large-vessel vasculitis

A
  • Takayasu arteritis

- Giant Cell (Temporal) arteritis

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7
Q

Name the 2 types of medium-vessel vasculitis

A
  • Polyarteritis nodosa (PAN)

- Kawasaki disease

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8
Q

Name 3 types of small-vessel vasculitis

A
  • Microscopic polyangiitis
  • Granulomatosis with polyangiitis (Wegener’s)
  • IgA vasculitis (Henoch-Schonlein)
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9
Q

Clinical presentation of large-vessel vasculitis

A
  • Limb claudication
  • Asymmetric BP (>10 mmHg diff.)
  • Absent pulses
  • Bruits
  • Aortic dilation
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10
Q

What BVs are affected in large vessel vasculitis?

A

Aorta and its major branches

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11
Q

What BVs are affected in medium vessel vasculitis?

A

Splenic, renal, hepatic, mesenteric arteries

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12
Q

Epidemiology of Takayasu arteritis

A

Pts <50 y/o

Typical pt is <40 y/o Asian woman

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13
Q

Clinical presentation of Takayasu arteritis

A
  • Constitutional sx’s
  • Large joint synovitis
  • HTN
  • Vascular sx’s uncommon in initial presentation
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14
Q

What artery is commonly involved in Takayasu arteritis?

A

Subclavian artery → can lead to subclavian steal syndrome

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15
Q

Dx Takayasu arteritis

A

Clinical featurs + imaging arterial tree by MRI, CT, or angiography

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16
Q

Labs in Takayasu arteritis

A
  • Normochromic normocytic anemia of chronic disease

- Elevated ESR, CRP

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17
Q

Tx acute Takayasu arteritis

A

Prednisone - max dose 60mg/day → taper over several weeks to lower tolerable dose

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18
Q

Tx chronic Takayasu arteritis

A

Angioplasty or bypass graft once irreversible arterial stenosis has occurred

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19
Q

What artery is commonly involved in giant cell arteritis?

A

Temporal artery

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20
Q

Giant cell arteritis is commonly associated w/ what disorder?

A

Polymyalgia rheumatica

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21
Q

Clinical presentation of giant cell arteritis

A
  • New H/A
  • Abrupt onset visual disturbances
  • Jaw claudication
  • Unexplained fever or anemia
  • Sx’s of polymyalgia rheumatica (shoulder/hip stiffness)
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22
Q

What labs would you get for giant cell arteritis?

A

ESR → always >70, usually >100

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23
Q

ACR criteria for diagnosing giant cell arteritis (5)

A
  • Age >50 y/o
  • New onset localized H/A
  • Tenderness or decreased pulse in temporal a.
  • ESR >50mm/hr
  • Biopsy revealing necrotizing arteritis w/ predominance of mononuclear cells or granulomatous process with multinucleated giant cells
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24
Q

Epidemiology of giant cell arteritis

A
  • Age >50

- Scandinavian descent (also Olmstead County, MN)

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25
Q

Gold standard for dx giant cell arteritis

A

Temporal artery biopsy

Note - Pts with Polymyalgia Rheumatica sx’s only (hip/shoulder stiffness) don’t need biopsy

26
Q

Most feared complication of giant cell arteritis is _______

A

Vision loss

27
Q

Tx giant cell arteritis w/out signs of vision loss (i.e. ischemic organ damage)

A

Prednisone + ASA

Taper initial 60mg/day prednisone 10% each week, until stopping at 12-16 months

28
Q

Tx giant cell arteritis w/ vision loss

A

IV methylprednisolone x3 days followed by PO steroids

29
Q

Epidemiology of polyarteritis nodosa

A

Incidence peaks in 50s

More common in males

30
Q

Polyarteritis nodosa is associated with what diseases?

A

HBV and HCV

31
Q

Clinical presentation of polyarteritis nodosa

A
  • Tends to spare lungs***
  • Constitutional sx’s
  • Peripheral neuropathies
  • HTN
  • Renal dysfunction
  • Purpura, livedo reticularis, ulcers, rashes
  • GIB, diarrhea
32
Q

What labs are relevant for polyarteritis nodosa?

A

ANCA negative

33
Q

ACR criteria for diagnosing polyarteritis nodosa

A

At least 3:

  • Unexplained weight loss >4kg
  • Livedo reticularis
  • Testicular pain
  • Myalgias, muscle weakness, polyneuropathy
  • New-onset diastolic >90mmHg
  • Elevated serum BUN
  • HBV
  • Characteristic arteriographic abnormalities
  • Bx small/medium-sized artery w/ polymorphonuclear cells
34
Q

Treatment of polyarteritis nodosa

A 
Mild = prednisone taper 
Moderate = prednisone + cyclphosphamide
Severe = IV methylprednisolone
35
Q

Epidemiology of Kawasaki disease

A

Infants & young children

36
Q

Main concern of Kawasaki disease

A

Coronary aneurysms if not treated w/ IVIG

37
Q

Dx Kawasaki disease

A

Fever >5 days with at least 4:

  • Bilateral conjunctival infection
  • Oral mucous membrane changes → strawberry tongue, injected pharynx, injected fissured lips
  • Peripheral extremity changes → erythema in palms/soles, edema in hands/feet, desquamation of skin
  • Polymorphous rash
  • Cervical LAN (>1.5cm)
38
Q

Diagnostics for Kawasaki disease

A

Baseline echo at diagnosis - repeat at weeks 2 and 6 to evaluate for CA involvement

39
Q

Tx Kawasaki disease

A

IVIG + aspirin + observation for 12-24 hrs to confirm fever resolution

40
Q

When is IVIG most effective for Kawasaki disease?

A

7-10 days

41
Q

Are glucocorticoids recommended for Kawasaki disease?

A

NO b/c doesn’t alter rate of coronary artery abnormalities

42
Q

Postpone live-virus vaccines for at least _______ in Kawasaki pts. Why?

A

11 months if received IVIG b/c IVIG can interfere w/ vaccine immunogenicity

43
Q

Exceptions for postponing live-virus vaccines in Kawasaki pts

A
  • Outbreak
  • Long-term aspirin therapy
  • 6+ months old with long term aspirin therapy should get varicella and inactivated influenza d/t Reye syndrome risk
44
Q

Microscopic polyangiitis is the most common cause of ________.

A

Pulmonary-renal syndrome

45
Q

Clinical presentation of microscopic polyangiitis

A
  • Purpura
  • Pulmonary hemorrhage
  • Ulcers
  • Splinter hemorrhages
46
Q

Labs for microscopic polyangiitis

A
  • ANCA positive

- Hematuria, proteinuria, RBC casts in urine

47
Q

Tx microscopic polyangiitis

A

Prednisone + cyclophosphamide

Recurrence is common

48
Q

Classic triad of Wegener’s (granulomatosis with polyangiitis)

A
  • Upper respiratory tract disease
  • Lower respiratory tract disease
  • Glomerulonephritis
49
Q

Is Wegener’s ANCA positive or negative?

A

ANCA positive

50
Q

Clinical presentation of Wegener’s

A
  • Crusting, ulceration, bleeding and/or perforation of nasal septum
  • Upper & lower respiratory tract sx’s
  • Proptosis, ptosis, ophthalmoplegia
  • Scleritis
51
Q

What is the best imaging for Wegener’s?

A

CT better choice than CXR b/c CXR may appear as lung cancer

52
Q

Treatment for Wegener’s

A

cyclophosphamide or rituximab + prednisone

53
Q

Henoch Schonlein is also known as ________

A

IgA vasculitis

54
Q

Epidemiology of Henoch Schonlein

A
  • Children

- Primarily occurs in fall, winter, spring

55
Q

Classic tetrad of Henoch Schonlein

A

Palpable purpura plus 1+:

  • Arthralgia/arthritis (usually in LE)
  • Abdominal pain
  • Renal disease
56
Q

Treatment of Henoch Schonlein

A
  • PO rehydration, rest, sx relief
  • NSAIDs for joint/abd pain
  • Prednisone if abd pain interferes w/ PO intake
57
Q

Follow up for Henoch Schonlein

A

Screen for urinary abnormalities and elevated BP d/t long-term renal complications

58
Q

What vessels are involve din Behcet’s disease?

A

Variable vessel vasculitis

59
Q

Classic triad of Behcet disease

A
  • Aphthous ulcers
  • Painful genital lesions
  • Recurrent eye inflammation (posterior/anterior uveitis)
60
Q

Is anterior or posterior uveitis more dangerous?

A

Posterior b/c fewer sx’s and can damage retina

61
Q

Besides the classic triad in Behcet disease, what else occurs?

A
  • Pulmonary artery aneurysms

- GI ulcerations

62
Q

Tx for Behcet’s disease

A

Steroids (max 60mg/day)

Clin Med IV - Ortho (7 decks)

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