Vasculitis Flashcards Preview

Clin Med IV - Ortho > Vasculitis > Flashcards

Flashcards in Vasculitis Deck (62):
1

When might you suspect vasculitis?

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

2

Vasculitis is associated with what autoimmune disorder?

SLE

3

Clinical presentation of vasculitis

- 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

4

________ + ________ should raise concern for vasculitis

Lung hemorrhage
Renal insufficiency
(Pulmonary-renal syndrome)

5

Standard imaging for screening for large-vessel vasculitis

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

6

Name the 2 types of large-vessel vasculitis

- Takayasu arteritis
- Giant Cell (Temporal) arteritis

7

Name the 2 types of medium-vessel vasculitis

- Polyarteritis nodosa (PAN)
- Kawasaki disease

8

Name 3 types of small-vessel vasculitis

- Microscopic polyangiitis
- Granulomatosis with polyangiitis (Wegener's)
- IgA vasculitis (Henoch-Schonlein)

9

Clinical presentation of large-vessel vasculitis

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

10

What BVs are affected in large vessel vasculitis?

Aorta and its major branches

11

What BVs are affected in medium vessel vasculitis?

Splenic, renal, hepatic, mesenteric arteries

12

Epidemiology of Takayasu arteritis

Pts <50 y/o
Typical pt is <40 y/o Asian woman

13

Clinical presentation of Takayasu arteritis

- Constitutional sx's
- Large joint synovitis
- HTN
- Vascular sx's uncommon in initial presentation

14

What artery is commonly involved in Takayasu arteritis?

Subclavian artery → can lead to subclavian steal syndrome

15

Dx Takayasu arteritis

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

16

Labs in Takayasu arteritis

- Normochromic normocytic anemia of chronic disease
- Elevated ESR, CRP

17

Tx acute Takayasu arteritis

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

18

Tx chronic Takayasu arteritis

Angioplasty or bypass graft once irreversible arterial stenosis has occurred

19

What artery is commonly involved in giant cell arteritis?

Temporal artery

20

Giant cell arteritis is commonly associated w/ what disorder?

Polymyalgia rheumatica

21

Clinical presentation of giant cell arteritis

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

22

What labs would you get for giant cell arteritis?

ESR → always >70, usually >100

23

ACR criteria for diagnosing giant cell arteritis (5)

- 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

24

Epidemiology of giant cell arteritis

- Age >50
- Scandinavian descent (also Olmstead County, MN)

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)