Lecture 11: Vasculitis

Question 1

• 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

Answer 1

• Large vessel = aorta and great vessels
• Medium vessel = splanchnic vessels (kidney/abd)
• Small vessel = capillaries, arterioles, and venules

Question 2

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

Answer 2

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

Also can frequently occur together.

Question 3

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

Answer 3

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

Question 4

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…

Answer 4

Polymyalgia rheumatica

Question 5

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

Answer 5

True

Question 6

The first-line tx for Isolated polymyalgia rheumatica is…

Answer 6

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

Can also add weekly MTX while using.

Usually an annual thing.

Question 7

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

Answer 7

ESR

Question 8

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

Answer 8

GCA/Temporal arteritis

Question 9

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

Answer 9

The aorta and its branches

Question 10

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

Answer 10

Adventitia

Question 11

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?

Answer 11

• GCA/temporal arteritis
• Concerned for her VISION!!!

Question 12

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

Answer 12

False. Normally its just normal

Question 13

Temporal arteritis is associated with:

• ESR ()
• (MCHC), (MCV) anemia
• Serum CK ()

Answer 13

• Elevated ESR (> 50 to > 100)
• Normochromic, normocytic anemia
• Serum CK is NORMAL

Question 14

The standard of diagnosis for GCA is…

Answer 14

Temporal artery biopsy

Question 15

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

Answer 15

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

Question 16

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

Answer 16

High dose prednisone for 1 month

Can add tocilizumab to reduce prednisone use.

Question 17

Tx of GCA w/ visual loss is…

Answer 17

IV methylprednisolone

Monitor via ESR.

Question 18

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

Answer 18

Steroid toxicity

Also can induce psychosis

Question 19

GCA greatly increases the risk of a () aneurysm

Answer 19

Thoracic aortic aneurysm

Question 20

Polymyalgia vs GCA

Answer 20

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

Question 21

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

Answer 21

Hep B

Question 22

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

Answer 22

Renal and visceral arteries

Question 23

One organ system that PAN does NOT affect is…

Answer 23

Lungs

Question 24

The characteristic finding of arteries in PAN is…

Answer 24

Aneurysmal dilation up to 1 cm.

Question 25

The hallmark presentation of PAN is...

Answer 25

Nonspecific symptoms. | FLS + extremity pain

Question 26

The MC skin finding seen in PAN is...

Answer 26

Lower extremity ulcerations near the malleoli

Question 27

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

Answer 27

Secondary renin-mediated HTN

Question 28

The diagnosis of PAN is made primarily with...

Answer 28

Tissue biopsies or angiogram | ANCA negative, no specific serologies.

Question 29

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

Answer 29

* High dose prednisone * IV methylprednisolone * Cyclophosphamide | Basically its like GCA tx

Question 30

**Remission** of PAN is maintained by...

Answer 30

MTX or azathioprine | But initial tx is prednisone

Question 31

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

Answer 31

* Anti-HBV therapy * Plasmapheresis

Question 32

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

Answer 32

GI or CV causes

Question 33

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

Answer 33

Blacks

Question 34

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

Answer 34

* Upper and lower respiratory tracts * Glomerulonephritis

Question 35

GPA primarily affects (big/medium/small) vessels

Answer 35

Small vessel vasculitis

Question 36

The classic triad of involvement with GPA is...

Answer 36

* Upper airway lesions * Lower airway (Lungs) * Renal

Question 37

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

Answer 37

* Chronic nasal staph aureus * Saddle nose deformity * Serous otitis media

Question 38

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

Answer 38

Pulmonary tissue (lower airway)

Question 39

T/F: ANCA positive = diagnosis of GPA

Answer 39

False.

Question 40

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

Answer 40

**Cyclophosphamide with prednisone for a month**, then taper down over 6-9 months. | The next slide with rituximab seems unapproved

Question 41

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 ()

Answer 41

* MTX (but cannot use in renal or liver dz) * Azathioprine | Last resort is mycophenolate mofetil

Question 42

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

Answer 42

MTX + prednisone

Question 43

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

Answer 43

Upper airway. | If it is outside of upper airway, cannot use alone

Question 44

The MCC of pulmonary-renal syndrome is...

Answer 44

Microscopic polyangiitis

Question 45

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

Answer 45

Non-granulomatous

Question 46

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 ()

Answer 46

* ANCA test, since PAN is not associated with ANCA. * Biopsy showing LACK of granulomatous inflammation

Question 47

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

Answer 47

Rituximab

Question 48

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

Answer 48

Renal function for MTX

Question 49

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

Answer 49

* MPA is usually more kidneys * GPA is usually more lungs (upper and lower airways)

Question 50

The MC vasculitis for children is...

Answer 50

Henoch shonlein purpura (HSP)

Question 51

The characteristic presentation of HSP is: * () on lower ext/butt * poly() * () pain * Glomerulonephritis

Answer 51

* Palpable purpura on lower ext/ass * Polyarthralgia * Abd pain * GN

Question 52

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

Answer 52

Nothing? Spontaneously resolves without tx. | More common in adults for this weird chronic course.

Question 53

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

Answer 53

Prednisone

Question 54

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

Answer 54

* Mycophenolate mofetil * Recovery is complete!

Question 55

ANCA is used primarily for what two vasculitis?

Answer 55

* GPA * MPA

Question 56

Which ANCA subtype is highly specific for GPA?

Answer 56

c-ANCA | BUT YOU DO NOT DIAGNOSE WITH c-ANCA