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Vasculitis > Ch. 34 Polyarteritis Nodosa > Flashcards

Ch. 34 Polyarteritis Nodosa Flashcards

1
Q

Definition of classical PAN

A

Necrotizing inflammation of medium- or small-sized arteries, without vasculitis in arterioles, capillaries, or venules

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2
Q

Definition of microscopic polyangiitis (MPA)

A

Necrotizing vasculitis with few or no immune deposits affecting small vessels

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3
Q

ANCA-associated vasculitis which is predominantly a renal disease and usually presents in childhood as RPGN

A

MPA

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4
Q

Classification criteria for childhood PAN

A

Mandatory histopath or angio + 1 out of 5:

  1. Skin involvement (livedo, nodules, or infarcts)
  2. Myalgia or muscle tenderness
  3. Htn
  4. Peripheral neuropathy
  5. Renal involvement
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5
Q

MC clinical manifestations of PAN

A

Cutaneous, fever, and myalgia

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6
Q

T/F ANCA and ANA are typically positive in PAN

A

F, negative

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7
Q

Most valuable imaging procedure for the diagnosis of PAN

A

Catheter digital subtraction angiography

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8
Q

Common finding in DSA indicative of PAN

A

Aneurysms

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9
Q

Most reliable nonaneurysmal signs on imaging that supports PAN

A

Perfusion defects
Collateral arteries
Delayed emptying of small renal arteries

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10
Q

GOLD STANDARD in the diagnosis of PAN

A

Conventional angiography

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11
Q

T/F Tissue biopsy should be performed in patient with suspected PAN

A

T

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12
Q

Characteristic histopath changes in PAN

A

Fibrinoid necrosis of walls of medium or small arteries with marked inflammatory response within or surrounding a vessel wall

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13
Q

T/F Absence of supportive histopathological findings excludes a diagnosis of PAN

A

F, disease is patchy in nature

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14
Q

T/F Glomerulonephritis is common in PAN

A

F, true GN is uncommon, and when present, suggests polyangiitis overlap

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15
Q

Treatment for mild PAN and isolated cutaneous disease

A

Monotherapy with oral glucocorticoid (Prednisone 1mkd) for 4 weeks. If with substantial improvement, taper slowly until 20mg/day (adult 0.3mkd if 60kg) is reached by ~3-4 months. Then decrease by 2.5mg every 14 days. Overall course of 6-8 months until d/c. If with inadequate response, (no response in 3 months or cannot be tapered to at least 10mg/day without relapse) add AZA (2mkd) or MTX (20-25mg weekly) to be given for at least 1 year following attainment of clinical remission.

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16
Q

Induction for moderate to severe PAN

A

Glucocorticoid (Pred 1mkd with or w/o IV MPPT depending on severity) + CYC (0,2,4,then every 3 weeks @ 15mg/kg OR 600mg/m2 every 2 weeks x 3 doses then every 4 weeks for at least 4 months until remission but no greater than 6 months.

17
Q

Maintenance for moderate to severe PAN

A

12-36 months. MMF, AZA, MTX. May be withdrawn slowly over at least 6 months if patient has been on remission for at least 12 months on maintenance therapy.

18
Q

T/F PAN appears to be a condition in which permanent remission can be achieved

A

T

19
Q

Important aspects of management that should be considered in patients with PAN

A
  1. Antiplatelet
  2. PCP prohpylaxis
  3. Osteoporosis prophylaxis
  4. Gastric protection
  5. GAS prophylaxis when implicated, added to induction therapy for at least 12 months
20
Q

Recommended approach to Hep B associated PAN

A

Plasma exchange +
Antiviral treatment +
Corticosteroids to control acute manifestations
THEN stop corticosteroids to enhance immunologic clearance of virus

21
Q

Characteristics of skin lesions in cutaneous PAN

A

Nodular, painful, nonpurpuric, with or without livedo racemosa, predominantly in the LE

22
Q

Systemic involvement that may be seen in cPAN

A

Myalgia, arthralgia, nonerosive arthritis

23
Q

Differentiates cPAN from PAN in terms of skin features

A

Infarcts are not present in cutaneous disease

24
Q

Differentiates PAN from cPAN in terms of clinical course

A

cPAN is characterized by periodic exacerbations and remissions

25
Q

Treatment for cPAN

A

NSAIDs and/or corticosteroids alone may be appropriate

26
Q

Organism implicated in PAN and cPAN for which prohylaxis may be needed for at least 1 year

A

GAS

27
Q

Mimic of PAN for which prompt anti-TNF treatment is warranted

A

DADA2

28
Q

Mimic of vasculitis that presents with an impressive livedo racemosa

A

DADA2, deficiency of adenosine deaminase 2

29
Q

Child can present with features of sJIA but with prominent CNS disease (recurrent strokes)

A

DADA2

Vasculitis (8 decks)

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