Lecture 11: Vasculitis Flashcards

1
Q
  • Large vessel vasculitis means it involves the () and () vessels.
  • Medium vessel vasculitis means it involves the () vessels
  • Small vessel vasculitis means it involves the (), () and () to lungs, kidneys, and skin
A
  • Large vessel = aorta and great vessels
  • Medium vessel = splanchnic vessels (kidney/abd)
  • Small vessel = capillaries, arterioles, and venules
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2
Q

Both polymyalgia rheumatica and temporal arteritis share preference for the same () haplotype and both MC occur after the age of ()

A
  • Same HLA haplotype.
  • Both occur after 50 usually.

Also can frequently occur together.

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3
Q

T/F: Polymyalgia rheumatica typically occurs with temporal arteritis.

A

False. It MC begins as polymyalgia rheumatica isolated and later develops temporal arteritis

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4
Q

Your patient has trouble combing their hair, putting on a coat, and difficulty rising out of a chair. It hurts and is stiff! There’s no swelling and their temporal artery looks fine. You suspect they have…

A

Polymyalgia rheumatica

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5
Q

T/F: ESR and CRP are elevated in polymyalgia rheumatica

A

True

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6
Q

The first-line tx for Isolated polymyalgia rheumatica is…

A

Prednisone daily over 2-4 weeks with a slow taper.

Can also add weekly MTX while using.

Usually an annual thing.

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7
Q

We use (ESR/CRP) to monitor progress of isolated polymyalgia rheumatica.

A

ESR

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8
Q

A 79+ year old scandinavian women with HLA-DR4 describes the classic demographic for what vasculitis?

A

GCA/Temporal arteritis

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9
Q

Although the temporal artery is the MC carotid branch affected in GCA, it can also spread to…

A

The aorta and its branches

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10
Q

What layer of a blood vessel is GCA/temporal arteritis suspected to begin within?

A

Adventitia

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11
Q

You have a 60F presenting with headache, scalp tenderness, and intermittent amaurosis fugax. She also notes jaw claudication. You believe she has (vasculitis), and you should be concerned for what organ system specifically?

A
  • GCA/temporal arteritis
  • Concerned for her VISION!!!
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12
Q

T/F: Temporal artery thickening/nodularity is the typical finding on PE for GCA.

A

False. Normally its just normal

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13
Q

Temporal arteritis is associated with:

  • ESR ()
  • (MCHC), (MCV) anemia
  • Serum CK ()
A
  • Elevated ESR (> 50 to > 100)
  • Normochromic, normocytic anemia
  • Serum CK is NORMAL
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14
Q

The standard of diagnosis for GCA is…

A

Temporal artery biopsy

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15
Q

T/F: Blindness is GCA once it occurs is usually reversible.

A

False. Once you turn blind blind in GCA, you’re done :(

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16
Q

The first-line pharm tx for non-visual loss GCA is…

A

High dose prednisone for 1 month

Can add tocilizumab to reduce prednisone use.

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17
Q

Tx of GCA w/ visual loss is…

A

IV methylprednisolone

Monitor via ESR.

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18
Q

Your patient being treated for GCA is starting to show symptoms of hyperglycemia and HTN. You suspect that this is due to…

A

Steroid toxicity

Also can induce psychosis

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19
Q

GCA greatly increases the risk of a () aneurysm

A

Thoracic aortic aneurysm

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20
Q

Polymyalgia vs GCA

A

I feel like jaw claudication and vision are the biggest things for GCA. Its the more severe disease.

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21
Q

() infection is seen in 10% of patients with Polyarteritis Nodosa

A

Hep B

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22
Q

Polyarteritis Nodosa (PAN) primarily affects the (organ) and () arteries, causing necrotizing arteritis.

A

Renal and visceral arteries

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23
Q

One organ system that PAN does NOT affect is…

A

Lungs

24
Q

The characteristic finding of arteries in PAN is…

A

Aneurysmal dilation up to 1 cm.

25
Q

The hallmark presentation of PAN is…

A

Nonspecific symptoms.

FLS + extremity pain

26
Q

The MC skin finding seen in PAN is…

A

Lower extremity ulcerations near the malleoli

27
Q

If PAN affects the renal artery, () HTN can occur.

A

Secondary renin-mediated HTN

28
Q

The diagnosis of PAN is made primarily with…

A

Tissue biopsies or angiogram

ANCA negative, no specific serologies.

29
Q

PAN is managed with (), but if critically ill, you should use (). Lastly, addition of () has a mortality benefit.

A
  • High dose prednisone
  • IV methylprednisolone
  • Cyclophosphamide

Basically its like GCA tx

30
Q

Remission of PAN is maintained by…

A

MTX or azathioprine

But initial tx is prednisone

31
Q

If a PAN pt is also HBV positive, you need to add () therapy and () along steroids

A
  • Anti-HBV therapy
  • Plasmapheresis
32
Q

Death from PAN is typically seen due to () or () causes

A

GI or CV causes

33
Q

Granulomatosis with Polyangiitis/Wegener’s/GPA is extremely rare in what ethnicity?

A

Blacks

34
Q

GPA is characterized by a vasculitis of the () tracts and ()itis

A
  • Upper and lower respiratory tracts
  • Glomerulonephritis
35
Q

GPA primarily affects (big/medium/small) vessels

A

Small vessel vasculitis

36
Q

The classic triad of involvement with GPA is…

A
  • Upper airway lesions
  • Lower airway (Lungs)
  • Renal
37
Q

You might suspect GPA in people with chronic (nose), (nose deformity), and (ear infection)

A
  • Chronic nasal staph aureus
  • Saddle nose deformity
  • Serous otitis media
38
Q

The diagnosis of () tissue in GPA has the highest diagnostic yield, showing necrotizing granulomatous vasculitis.

A

Pulmonary tissue (lower airway)

39
Q

T/F: ANCA positive = diagnosis of GPA

A

False.

40
Q

The management of severe GPA is via () with ()

A

Cyclophosphamide with prednisone for a month, then taper down over 6-9 months.

The next slide with rituximab seems unapproved

41
Q

Once remission is obtained with cyclophosphamide in GPA, you must switch it off to a different med after 3-6 months, and the options are () or ()

A
  • MTX (but cannot use in renal or liver dz)
  • Azathioprine

Last resort is mycophenolate mofetil

42
Q

In non-severe GPA, you can instead use () with () to begin management/induce remission.

A

MTX + prednisone

43
Q

Bactrim can be used for GPA as sole treatment, provided GPA is only affecting the ()

A

Upper airway.

If it is outside of upper airway, cannot use alone

44
Q

The MCC of pulmonary-renal syndrome is…

A

Microscopic polyangiitis

45
Q

Although microscopic polyangiitis and GPA affect both pulm and renal small/medium vessels, the main difference lies in that microscopic polyangiitis is ()

A

Non-granulomatous

46
Q

Your patient presents with pulmonary and renal issues like ILD and glomerulonephritis. Their nails also show splinter hemorrhages and they have palpable raised purpura. You suspect this is microscopic polyangiitis. HOWEVER, to provide it is not PAN, you should obtain a () lab test. To prove it is not GPA, you should obtain a ()

A
  • ANCA test, since PAN is not associated with ANCA.
  • Biopsy showing LACK of granulomatous inflammation
47
Q

MPA and GPA are treated pretty much the same anyways, but instead of cyclophosphamide for induction tx, you can use ()

A

Rituximab

48
Q

You are deciding between azathioprine and MTX for remission maintenance in MPA. What organ function must be checked and for which drug?

A

Renal function for MTX

49
Q

MPA affects the (organ) more, while GPA affects (organ) more.

A
  • MPA is usually more kidneys
  • GPA is usually more lungs (upper and lower airways)
50
Q

The MC vasculitis for children is…

A

Henoch shonlein purpura (HSP)

51
Q

The characteristic presentation of HSP is:

  • () on lower ext/butt
  • poly()
  • () pain
  • Glomerulonephritis
A
  • Palpable purpura on lower ext/ass
  • Polyarthralgia
  • Abd pain
  • GN
52
Q

If HSP occurs with just intermittent purpura and no other symptoms, we would manage it via ()

A

Nothing? Spontaneously resolves without tx.

More common in adults for this weird chronic course.

53
Q

In children, the first-line pharm tx for normal HSP is…

A

Prednisone

54
Q

Severe HSP is treated with () and recovery is usually ()

A
  • Mycophenolate mofetil
  • Recovery is complete!
55
Q

ANCA is used primarily for what two vasculitis?

A
  • GPA
  • MPA
56
Q

Which ANCA subtype is highly specific for GPA?

A

c-ANCA

BUT YOU DO NOT DIAGNOSE WITH c-ANCA