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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
Gold standard for dx giant cell arteritis
Temporal artery biopsy Note - Pts with Polymyalgia Rheumatica sx's only (hip/shoulder stiffness) don't need biopsy
26
Most feared complication of giant cell arteritis is _______
Vision loss
27
Tx giant cell arteritis w/out signs of vision loss (i.e. ischemic organ damage)
Prednisone + ASA Taper initial 60mg/day prednisone 10% each week, until stopping at 12-16 months
28
Tx giant cell arteritis w/ vision loss
IV methylprednisolone x3 days followed by PO steroids
29
Epidemiology of polyarteritis nodosa
Incidence peaks in 50s | More common in males
30
Polyarteritis nodosa is associated with what diseases?
HBV and HCV
31
Clinical presentation of polyarteritis nodosa
- Tends to spare lungs*** - Constitutional sx's - Peripheral neuropathies - HTN - Renal dysfunction - Purpura, livedo reticularis, ulcers, rashes - GIB, diarrhea
32
What labs are relevant for polyarteritis nodosa?
ANCA negative
33
ACR criteria for diagnosing polyarteritis nodosa
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
Treatment of polyarteritis nodosa
``` Mild = prednisone taper Moderate = prednisone + cyclphosphamide Severe = IV methylprednisolone ```
35
Epidemiology of Kawasaki disease
Infants & young children
36
Main concern of Kawasaki disease
Coronary aneurysms if not treated w/ IVIG
37
Dx Kawasaki disease
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
Diagnostics for Kawasaki disease
Baseline echo at diagnosis - repeat at weeks 2 and 6 to evaluate for CA involvement
39
Tx Kawasaki disease
IVIG + aspirin + observation for 12-24 hrs to confirm fever resolution
40
When is IVIG most effective for Kawasaki disease?
7-10 days
41
Are glucocorticoids recommended for Kawasaki disease?
NO b/c doesn't alter rate of coronary artery abnormalities
42
Postpone live-virus vaccines for at least _______ in Kawasaki pts. Why?
11 months if received IVIG b/c IVIG can interfere w/ vaccine immunogenicity
43
Exceptions for postponing live-virus vaccines in Kawasaki pts
- 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
Microscopic polyangiitis is the most common cause of ________.
Pulmonary-renal syndrome
45
Clinical presentation of microscopic polyangiitis
- Purpura - Pulmonary hemorrhage - Ulcers - Splinter hemorrhages
46
Labs for microscopic polyangiitis
- ANCA positive | - Hematuria, proteinuria, RBC casts in urine
47
Tx microscopic polyangiitis
Prednisone + cyclophosphamide Recurrence is common
48
Classic triad of Wegener's (granulomatosis with polyangiitis)
- Upper respiratory tract disease - Lower respiratory tract disease - Glomerulonephritis
49
Is Wegener's ANCA positive or negative?
ANCA positive
50
Clinical presentation of Wegener's
- Crusting, ulceration, bleeding and/or perforation of nasal septum - Upper & lower respiratory tract sx's - Proptosis, ptosis, ophthalmoplegia - Scleritis
51
What is the best imaging for Wegener's?
CT better choice than CXR b/c CXR may appear as lung cancer
52
Treatment for Wegener's
cyclophosphamide or rituximab + prednisone
53
Henoch Schonlein is also known as ________
IgA vasculitis
54
Epidemiology of Henoch Schonlein
- Children | - Primarily occurs in fall, winter, spring
55
Classic tetrad of Henoch Schonlein
Palpable purpura plus 1+: - Arthralgia/arthritis (usually in LE) - Abdominal pain - Renal disease
56
Treatment of Henoch Schonlein
- PO rehydration, rest, sx relief - NSAIDs for joint/abd pain - Prednisone if abd pain interferes w/ PO intake
57
Follow up for Henoch Schonlein
Screen for urinary abnormalities and elevated BP d/t long-term renal complications
58
What vessels are involve din Behcet's disease?
Variable vessel vasculitis
59
Classic triad of Behcet disease
- Aphthous ulcers - Painful genital lesions - Recurrent eye inflammation (posterior/anterior uveitis)
60
Is anterior or posterior uveitis more dangerous?
Posterior b/c fewer sx's and can damage retina
61
Besides the classic triad in Behcet disease, what else occurs?
- Pulmonary artery aneurysms | - GI ulcerations
62
Tx for Behcet's disease
Steroids (max 60mg/day)

Clin Med IV - Ortho (7 decks)

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