Trigger - Vasculitis

Question 1

HLA halpotype in pts >50 y/o

Answer 1

polymyalgia rheumatica and temporal arteritis

Question 2

pain/stiffness in neck, shoulders, lower back hips and thighs leading to provlems with ADLs such as combing hair, putting on coats and rising from chairs

Answer 2

polymyalgia rheumatica

Question 3

this increases risk of thoracic aortic aneurysm by 17-18x

Answer 3

temporal arteritis (GCA)

Question 4

what is the cause of blindness in temporal arteritis (GCA)

Answer 4

anterior ischemic optic neuropathy

Question 5

Elevated ESR
thrombocytosis
elevated alkaline phosphatase
normochromic normocytic anemia

Answer 5

temporal arteritis (GCA)

Question 6

what are the 3 medications we learned about that can potentially be used in the tx of temporal arteritis (GCA) w no visual loss

Answer 6

• high dose prednisone x 1 month
• ASA to reduce stroke/vision loss
• tocilizumab (reduces prolonged use of prednisone)

Question 7

10% of people with this disease are found to have Hep B infections

Answer 7

Polyarteritis Nodosa (PAN)

Question 8

primarily affects the renal and visceral arteries causing nectrotizing arteritis

Answer 8

PAN

Question 9

Does polyarteritis cause muscular weakness?

Answer 9

NO!!

Question 10

This diagnosis spares the lungs but CAN affect the bronchial vessels

Answer 10

PAN

Question 11

aneurysmal dilation up to 1cm in involved arteries is characteristic for…

Answer 11

PAN

Question 12

Just look at this pathophys

Answer 12

Question 13

Presents with levido reticularis, uclers on the malleoli, and gangrene of the digits

Answer 13

PAN

Will also present with flu like symptoms!!!

Question 14

Flu like symptoms is the hallmark presenttion for this

Answer 14

PAN

Question 15

Associated with secondary renin-mediated hypertension

Answer 15

PAN with renal artery involvement

Question 16

Negative ANCA

Answer 16

PAN

GPA and MPA are positive!

Question 17

what medication in PAN treatment has morality benefit

Answer 17

cyclophosphamide

tx with high dose prednisone, IV if severe.

Question 18

Just tell me how to treat PAN

Answer 18

1. high dose CS (60mg/day)
2. IV methylprednisone if severe!
3. add cyclophosphamide to reduce mortality
4. If HBV+ then use anti-HBV and plasmapharesis
5. Once in remission use azothioprine or MTX to maintain remission

Question 19

what are the poor prognostic factors for PAN and how much do they reduce mortality

Answer 19

• CKD w Cr>1.6
• Proteinuria >1g/day
• GI ischemia
• CNS disease
• Cardio involvement

Question 20

characterizied by vasculitis of the upper and lower respiratory tracts with glomerulonephritis

Answer 20

Granulomatosis w polyangitis (GPA)

classic triad!!

Question 21

saddle nose deformity, serous otitis media, and chronic nasal staph aureus are all associated with what diagnosis

Answer 21

Granulomatosis w polyangitis (GPA)

Question 22

clinical presenttion includes severe upper respiratory problems with skin lesions, conjunctivitis, and possible severe airway obstruction

Answer 22

Granulomatosis w polyangitis (GPA)

this is alot:(

Question 23

pulmonary tissue biopsy has the highest diagnostic yield for this disease

Answer 23

Granulomatosis w polyangitis (GPA)

can also use renal biopsy to confirm pauci immune glomerulonephritis (idk seems important)

Question 24

labs show elevated ESR, leukocytosis, mild hypergammaglobulinemia, and mild elevation of RF

also thrombocytosis

Answer 24

Granulomatosis w polyangitis (GPA)

will also see + ANCA but you KNOW THAT!!!

Question 25

How do you treat severe Granulomatosis w polyangitis (GPA)

Answer 25

* cyclophosphamide + prednisone x1 month * continue prednisone x 6-9 mo * CBC Q 2 weeks * Rituximab * MTX or azathiprine to maintain remission (if cant take these then use mycophenolate mofetil) | I think you do all of these in this order but idk help plz

Question 26

How do you treat non-severe Granulomatosis w polyangitis (GPA)

Answer 26

MTX + glucocorticoid | like if its not life threatening

Question 27

if GPA is confined to the upper airway and is NOT found anywhere else, what can be used as treatment

Answer 27

bactrim. dont use alone if GPA is outside upper airway

Question 28

What are the concerns with treating GPA w cyclophosphamide

Answer 28

* renally eliminated (monitor kidney fxn) * infertility * DVT and PE risk * NEVER irradiate upper airway lesions!

Question 29

what is the MCC of pulmonary-renal syndrome

Answer 29

Microscopic polyangiitis (MPA) causes diffuse alveolar hemorrhaging + glomerulonephritis that often occurs simultaneously

Question 30

how do you differentiate GPA and MPA

Answer 30

biopsy showing granulomatous inflammation = GPA if Lack of granulomatous inflamm = MPA:)

Question 31

What is the pattern of ANCA followed by MPA

Answer 31

p-ANCA! | I think GPA = c-ANCA

Question 32

what medication must have normal renal function in order to use?

Answer 32

MTX

Question 33

leucocytoclastic vasculitis with IgA depostition

Answer 33

henock Shonlein purpura (HSP)

Question 33

what is the treatment of henock Shonlein purpura (HSP) in children with chronic persistent or intermittent type | what is the catch with this treatment

Answer 33

prednisone to decrease tissue edema, arthralgias and abdominal discomfort DOES NOT decrease proteinuria or shorten/decrease chance of recurrence.

Question 34

Tx of severe henock Shonlein purpura (HSP)

Answer 34

* mycophenolate mofetil