Trigger - Vasculitis Flashcards

1
Q

HLA halpotype in pts >50 y/o

A

polymyalgia rheumatica and temporal arteritis

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2
Q

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

A

polymyalgia rheumatica

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3
Q

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

A

temporal arteritis (GCA)

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4
Q

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

A

anterior ischemic optic neuropathy

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5
Q

Elevated ESR
thrombocytosis
elevated alkaline phosphatase
normochromic normocytic anemia

A

temporal arteritis (GCA)

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6
Q

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

A
  • high dose prednisone x 1 month
  • ASA to reduce stroke/vision loss
  • tocilizumab (reduces prolonged use of prednisone)
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7
Q

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

A

Polyarteritis Nodosa (PAN)

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8
Q

primarily affects the renal and visceral arteries causing nectrotizing arteritis

A

PAN

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9
Q

Does polyarteritis cause muscular weakness?

A

NO!!

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10
Q

This diagnosis spares the lungs but CAN affect the bronchial vessels

A

PAN

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11
Q

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

A

PAN

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12
Q

Just look at this pathophys

A
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13
Q

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

A

PAN

Will also present with flu like symptoms!!!

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14
Q

Flu like symptoms is the hallmark presenttion for this

A

PAN

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15
Q

Associated with secondary renin-mediated hypertension

A

PAN with renal artery involvement

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16
Q

Negative ANCA

A

PAN

GPA and MPA are positive!

17
Q

what medication in PAN treatment has morality benefit

A

cyclophosphamide

tx with high dose prednisone, IV if severe.

18
Q

Just tell me how to treat PAN

A
  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
19
Q

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

A
  • CKD w Cr>1.6
  • Proteinuria >1g/day
  • GI ischemia
  • CNS disease
  • Cardio involvement
20
Q

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

A

Granulomatosis w polyangitis (GPA)

classic triad!!

21
Q

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

A

Granulomatosis w polyangitis (GPA)

22
Q

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

A

Granulomatosis w polyangitis (GPA)

this is alot:(

23
Q

pulmonary tissue biopsy has the highest diagnostic yield for this disease

A

Granulomatosis w polyangitis (GPA)

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

24
Q

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

also thrombocytosis

A

Granulomatosis w polyangitis (GPA)

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

25
Q

How do you treat severe Granulomatosis w polyangitis (GPA)

A
  • 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

26
Q

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

A

MTX + glucocorticoid

like if its not life threatening

27
Q

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

A

bactrim. dont use alone if GPA is outside upper airway

28
Q

What are the concerns with treating GPA w cyclophosphamide

A
  • renally eliminated (monitor kidney fxn)
  • infertility
  • DVT and PE risk
  • NEVER irradiate upper airway lesions!
29
Q

what is the MCC of pulmonary-renal syndrome

A

Microscopic polyangiitis (MPA)

causes diffuse alveolar hemorrhaging + glomerulonephritis that often occurs simultaneously

30
Q

how do you differentiate GPA and MPA

A

biopsy showing granulomatous inflammation = GPA

if Lack of granulomatous inflamm = MPA:)

31
Q

What is the pattern of ANCA followed by MPA

A

p-ANCA!

I think GPA = c-ANCA

32
Q

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

A

MTX

33
Q

leucocytoclastic vasculitis with IgA depostition

A

henock Shonlein purpura (HSP)

33
Q

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

what is the catch with this treatment

A

prednisone to decrease tissue edema, arthralgias and abdominal discomfort

DOES NOT decrease proteinuria or shorten/decrease chance of recurrence.

34
Q

Tx of severe henock Shonlein purpura (HSP)

A
  • mycophenolate mofetil