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Rheum > 02b: Vasculitis > Flashcards

02b: Vasculitis Flashcards

1
Q

List some serologic tests used to distinguish various forms of vasculitis.

A
  1. ANCA
  2. Hep (B/C)
  3. Circulating cryoglobulins
  4. anti-GBM Ab
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2
Q

List the “Red Flags” of vasculitis.

A

Mnemonic: PIGSFEL

  1. Purpura/petechiae
  2. Ischemia/infarct of bowel
  3. Glomerulonephritis (rapid)
  4. Stroke (unexplained)
  5. Fever (unknown origin)
  6. Eosinophilia
  7. Livido reticularis
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3
Q

T/F: There is no single diagnostic test for vasculitis.

A

True - combo of clinical, serological, pathological evidence

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4
Q

“Small” vessel vasculitis includes which structures?

A

Arteriole, cap, venule

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5
Q

“Medium” vessel vasculitis includes which structures?

A

Arteries

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6
Q

“Large” vessel vasculitis includes which structures?

A

Aorta or primary branch vessel

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7
Q

List the Large vessel vasculitides.

A
  1. GCA

2. Takayasu’s arteritis

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8
Q

List the Medium vessel vasculitides.

A
  1. Polyarteritis nodosa (PAN)

2. Kawasaki’s disease

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9
Q

Purpura is a characteristic feature of (small/med/large) vessel vasculitis.

A

Small

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10
Q

(X) disease, a (small/med/large) vessel vasculitis, is associated with coronary artery aneurysms.

A

X = Kawasaki’s

Medium

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11
Q

Alveolar hemorrhages are characteristic of (small/med/large) vessel vasculitis. And scleritis?

A

Both small vessel

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12
Q

GCA is a vasculitis of (old/young) people with (X) as the most common presenting symptom.

A

Old (over 50 and esp over 70);

X = headache (unilateral)

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13
Q

(X) is the most feared complication of GCA.

A

X = visual impairment and progression to full monocular blindness

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14
Q

Jaw claudication is a moderately specific symptom for (X) (small/med/large) vessel vasculitis.

A

X = GCA

Large vessel

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15
Q

Polymyalgia rheumatica is an inherent part of (X) vasculitis. How does this manifest clinically?

A

X = GCA

Shoulder and/or hip girdle arthralgia

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16
Q

How is GCA commonly diagnosed?

A

Vasculitis (granulomatous inflammation with multi-nucleated giant cells) found on temporal artery biopsy

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17
Q

Which lab findings are commonly found in GCA?

A
  1. Elevated ECR and CRP
  2. Anemia
  3. Thrombocytosis
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18
Q

Treatment of GCA always involves (X). Recurrent disease is (common/uncommon).

A

X = high-dose glucocorticoids

Common

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19
Q

Takayasu’s Arteritis generally seen in patients at (X) age, with the vast majority being (M/F) and (Y) ethnicity.

A

X = under 50 y.o.
Female
Y = asian

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20
Q

Takayasu’s Arteritis can be asymptomatic initially, but there is ongoing (X).

A

X = arterial stenosis

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21
Q

30 year old female with 4 months fatigue/light-headedness. You are unable to feel right radial pulse. Which vasculitis comes to mind?

A

Takayasu’s Arteritis

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22
Q

Takayasu’s Arteritis patient presents with (hypo/hyper)-tension. Why?

A

Hypertension; renal a stenosis

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23
Q

In addition to clinical findings, which imaging technique is used to diagnose Takayasu’s Arteritis?

A

Angiogram

24
Q

Takayasu’s Arteritis is particularly difficult to treat because:

A

Requires high-dose glucocorticoids for extended period of time (and patients usually young)

25
Q

(X) is the classic form of medium-vessel vasculitis. It can be divided into which two categories?

A

X = polyarteritis nodosa (PAN)

  1. Hepatitis-associated
  2. Idiopathic
26
Q

Patient presents with testicular tenderness and a serum positive anti-Hep B Ab. You suspect (X) vasculitis and would expect his (SBP/DBP) to be (Y).

A

X = polyarteritis nodosa (medium-vessel)

DBP over 90 mmHg (HT)

27
Q

Arteriographic abnormality in polyarteritis nodosa would show:

A

Aneurysm/occlusion of visceral arteries

28
Q

Patient presents with polyneuropathy and a mottled reticular pattern over both legs. If this patient also had significant weight (loss/gain), you would suspect (X) vasculitis.

A

Loss;

X = polyarteritis nodosa

29
Q

Histo changes in polyarteritis nodosa would show:

A

Transmural inflmmation of the arterial wall with fibrinoid necrosis

30
Q

Polyarteritis nodosa can typically involve (X) organs.

A

X = all (esp renal, visceral) except lungs

31
Q

T/F: Treatment of Polyarteritis nodosa includes high-dose glucocorticoids.

A

True

32
Q

GCA is diagnosed if 3/5 of which criteria are present?

A
  1. Age onset over 50
  2. Elevated ESR
  3. Temporal a abnormality
  4. New HA
  5. Abnormal a biopsy
33
Q

T/F: Polymyalgia Rheumatica is, by definition, seen in patients over 50

A

True

34
Q

T/F: Polymyalgia Rheumatica is likely causing patient’s stiffness/muscle weakness if steroids provide little to no relief.

A

False - PMR has remarkable response to steroids

35
Q

In addition to GCA, (X) disease affects the eye with ocular inflammation potentially resulting in blindness. What other symptoms might the patient have to clue you in to (X) as opposed to GCA?

A

X = Behcet’s

Painful oral/genital ulcers, skin lesions (erythema nodosum)

36
Q

List the forms of (large/med/small) vessel vasculitis associated with ANCA.

A
  1. Wegener’s granulomatosis (WG) aka Granulomatosis with polyangiitis (GPA)
  2. Microscopic polyangiitis (MPA)
  3. Churg-Strauss Syndrome (CSS)
37
Q

T/F: All patients with ANCA-associated vasculitis will test positive for ANCA.

A

False

38
Q

ANCA testing is high in (sensitivity/specificity).

A

Specificity (but sensitivity is low - not all with disease will test positive)

39
Q

ANCA testing step 1 involves (X). What are the two patterns seen?

A

X = Immunofluorescence

Cytoplasmic (C-ANCA) or Perinuclear (P-ANCA)

40
Q

ANCA testing step 2 involves (X). Which results are diagnostic for vasculitis?

A

X = Ag-specific ELISA (confirm specific auto-Ab)

ONLY Ab to:

  1. Proteinase 3 (PR3)/cANCAor
  2. Myeloperoxidase (MPO)/pANCA
41
Q

Wegener’s/GPA is associated with (c/p)ANCA positivity.

A 
Mainly cANCA (85%);
pANCA (15%)
42
Q

MPA vasculitis is associated with (c/p)ANCA positivity.

A

pANCA (over 90%!)

cANCA (under 10%)

43
Q

Churg Strauss is associated with (c/p)ANCA positivity.

A

pANCA (80%)

44
Q

Which ANCA-associated vasculitis has the lowest frequency of ANCA positivity?

A

Churg Strauss (40%)

45
Q

(X) ANCA-associated vasculitis has the most widespread clinical manifestations. Most commonly:

A

X = Wegener’s (GPA)

  1. Granulomatous inflammation of URT (esp nose/sinuses)
  2. Granulomatous disease of lungs/alveoli
  3. Rapidly progressive glomerulonephritis
46
Q

(X) is an exclusively small-vessel vasculitis with particular predilection for rapidly progressive glomerulonephritis and alveolar hemorrhage. Patients may initially appear to have (X), but “evolve” to more obviously have (Y) vasculitis.

A 
X = microscopic polyangiitis (MPA)
Y = GPA (Wegener's)

MPA has a more narrow set of manifestations than GPA

47
Q

You suspect either MPA or GPA as the vasculitis present in your patient. Biopsy shows granulomatous lesions. Which disease is it?

A

GPA

48
Q

You suspect either MPA or GPA as the vasculitis present in your patient. Labs show presence of cANCA. Which disease is it?

A

Could be either, but way more likely GPA

49
Q

(X) vasculitis includes the classic combo of asthma, pulmonary infiltrates, and (Y).

A 
X = Churg Strauss (CSS) aka Eosinophilic Granulomatosis with Polyangitis
Y = eosinophilia
50
Q

T/F: 90% of patients with Churg-Strauss have some form of asthma.

A

True

51
Q

Churg-Strauss patients with positive ANCA are more likely to have (X).

A

X = glomerulonephritis

52
Q

T/F: Unlike GPA/MPA, glomerulonephritis and pulmonary hemorrhages are uncommon in Churg-Strauss

A

True

53
Q

Churg-Strauss (EGPA) organ involvement: what are the two most frequent?

A
  1. Peripheral neuropathy (66-78%)

2. Skin (51-81%)

54
Q

While (X) is the mainstay of therapy for vasculitis, list other treatments that have been used.

A

X = Glucocorticoids

Cyclophosphamide, Azothiaprine, MTX, biologics

55
Q

List two main hematological abnormalities associated with SLE.

A
  1. Hemolytic anemia

2. Lupus anticoagulant (misnomer, since it’s a hypercoagulable state)

56
Q

SLE patients with renal involvement commonly fall into which class?

A

Class IV (diffuse proliferative)

Rheum (6 decks)

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