Vasculitis

Question 1

What is the treatment for most types of vasculitis?

Answer 1

• corticosteroids +/- other immunosuppressive agents

Question 2

A young female of asian descent who presented with a flulike illness at first has now returned to clinic complaining of claudication of the upper extremities. What is the diagnosis?

Answer 2

Takayasu Arteritis

Question 3

How is Takayasu arteritis diagnosed?

Answer 3

• clinical and imaging

* don’t biopsy d/t large vessels*

Question 4

What is the superficial temporal artery a branch of?

• internal carotid
• external carotid
• occipital artery
• ophthalmic artery
• superficial thyroid artery

Answer 4

• external carotid

Question 5

What is the treatment for giant cell arteritis?

Answer 5

prolonged course steroids, started immediately

start treatment before confirmation because you can have irreversible vision loss

Question 6

An old male patient presents with an abrupt onset of visual disturbances and headaches. Labs demonstrated a highly elevated ESR. What is the diagnosis?

Answer 6

Giant Cell arteritis

Question 7

How is giant cell arteritis diagnosed?

Answer 7

temporal artery biopsy

Question 8

What is polymyalgia rheumatica?

What vasculitis is it associated with?

Answer 8

• Chronic anti-inflammatory disorder characterized by symmetric aching and morning stiffness in the shoulder and hip girdle
• associated with giant cell arteritis
• in a patient with PMR always ask do they also have GCA?*

Question 9

How is polymyalgia rheumatica (PMR) managed?

Answer 9

low-dose daily prednisone

Question 10

A 60 yo patient with a PMHx of Hep B presents with fever, abdominal pain, and unexplained acute kidney injury. The abdominal CT demonstrates aneurysms of the blood vessels in the kidney. What is the diagnosis?

Answer 10

polyarteritis nodosa

Question 11

What is the feared complication of Kawasaki disease?

Answer 11

coronary artery aneurysms

cardiac testing is indicated early

Question 12

A child presents with prolonged fever, cracked lips, and a strawberry tongue. What type of vasculitis is this?

Answer 12

Kawasaki Disease

Question 13

Lower extremity purpuric rash that is palpable is consistent with which of the following:

a. small vessel vasculitis
b. medium vessel vasculitis
c. large vessel vasculitis

Answer 13

a. small vessel vasculitis

Question 14

A patient presents with chronic sinusitis and hemoptysis. On PE you notice a saddle deformity of the nose. Labs are positive for c-ANCA. The biopsy demonstrates necrotizing vasculitis with granulomas, but without immune complex deposits. What is the diagnosis?

a. granulomatosis with polyangiitis
b. eosinophilic granulomatosis with polyangiitis
c. microscopic polyangiitis
d. IgA Vasculitis
e. Cryoglobulinemic vasculitis

Answer 14

a. granulomatosis with polyangiitis

previously known as Wegener’s

Question 15

A patient with a PMHx of asthma presents with chronic sinusitis. Labs are positive for p-ANCA. The biopsy demonstrates necrotizing vasculitis with granulomas, but without immune complex deposits. What is the diagnosis?

a. granulomatosis with polyangiitis
b. eosinophilic granulomatosis with polyangiitis
c. microscopic polyangiitis
d. IgA Vasculitis
e. Cryoglobulinemic vasculitis

Answer 15

b. eosinophilic granulomatosis with polyangiitis

* difference between GPA and e-GPA is the antibodies*

Question 16

A patient presents with glomerulonephritis. On PE you notice palpable purpura. Labs are positive for p-ANCA. The biopsy demonstrates necrotizing vasculitis WITHOUT immune complex deposits or granuloma. What is the diagnosis?

a. granulomatosis with polyangiitis
b. eosinophilic granulomatosis with polyangiitis
c. microscopic polyangiitis
d. IgA Vasculitis
e. Cryoglobulinemic vasculitis

Answer 16

c. microscopic polyangiitis

Question 17

A child presents with palpable purpura and associated abdominal pain with bloody stool. Labs are negative for ANCA. The biopsy demonstrates IgA immune deposits. What is the most likely diagnosis?

Answer 17

IgA Vasculitis

Question 18

A patient with a PMHx of Hep C presents with palpaple purpura and joint pain. He notes this symptoms are worse with cold weather. Labs are negative for ANCA. The biopsy demonstrates cryoglobulin immune deposits and circulating serum cryoglobulin. What is the diagnosis?

Answer 18

Cryoglobulinemic vasculitis

Question 19

A patient of Turkish descent presents with recurrent, painful mucocutaneous ulcers of the mouth and scrotum. He also has associated uveitis and abdominal pain with n/v. What is the diagnosis?

Answer 19

Behcet Syndrome

Question 20

Behcet syndrome involves what type of vessel?

Answer 20

any type of vessel!

Question 21

A 5-year-old patient presents with a sore throat, sandpaper rash, and a “strawberry tongue”. What is the most likely diagnosis?

Answer 21

Scarlet Fever!

Question 22

Clinical manifestations of vasculitis are confirmed with ________.

Answer 22

biopsy

Question 23

What CXR findings is commonly seen in granulomatosis with polyangiitis?

Answer 23

cavitary lesions

Question 24

Which small vessel vasculitis is associated with cANCA?

GPA (Wegener’s)
EGPA (CSS)
MPA
Cryoglobulinemia
IgAV (HSP)

Answer 24

GPA (Wegener’s)

Question 25

Which small vessel vasculitis is associated with pANCA and necrotizing granulomas?

GPA (Wegener’s)
EGPA (CSS)
MPA
Cryoglobulinemia
IgAV (HSP)

Answer 25

EGPA (CSS)

Question 26

Which small vessel vasculitis is associated with pANCA, but NOT necrotizing granulomas?

GPA (Wegener’s)
EGPA (CSS)
MPA
Cryoglobulinemia
IgAV (HSP)

Answer 26

MPA (microscopic polyangiitis)

Question 27

Which small vessel vasculitis is associated with no ANCA, but cryoglobulin immune deposits?

GPA (Wegener’s)
EGPA (CSS)
MPA
Cryoglobulinemia
IgAV (HSP)

Answer 27

Cryoglobulinemia

Question 28

Which small vessel vasculitis is associated with no ANCA, but with IgA immune deposits?

GPA (Wegener’s)
EGPA (CSS)
MPA
Cryoglobulinemia
IgAV (HSP)

Answer 28

IgAV (HSP)

Question 29

Aneurysms of what artery are you concerned with in Behcet syndrome?

Answer 29

pulmonary arteries