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RMSK > 16: Medium & Large Vessel Vasculitis > Flashcards

16: Medium & Large Vessel Vasculitis Flashcards

1
Q

What are the types of medium and large vessel vasculites?

A
  • Medium vessel:
    • Polyarteritis nodosa
    • Kawasaki disease
  • ​Large vessel:
    • Giant cell arteritis
      • ​Polymyalgia rheumatica
    • ​Takayasu’s arteritis
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2
Q

What is polyarteritis nodosa (PAN)?

A

Multisystem, necrotizing vasculitis of small and medium-sized muscular arteries in which involvement of the renal and visceral arteries is characteristic; does not involve pulmonary arteries, although bronchial vessels may be involved; granulomas, significant eosinophilia, and an allergic diathesis are not observed

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3
Q

PAN affected vasculature

A

Primarily below the diaphragm

  • SMA
  • Celiac & hepatic arteries
  • Renal arteries
  • Musclar arteries of extremities
  • Coronary arteries (uncommon)
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4
Q

PAN epidemiology

A
  • 20-70yo
  • No racial/ethnic predilection
  • Incidence:
    • 2-4/1,000K annually in US
    • 70-80/1,000K annually in regions endemic for HBV
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5
Q

What is the association between PAN and HBV?

A

PAN occurs acutely in first 6mos following HBV infx; usually + for surface and e antigens

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6
Q

What does this image show?

A

Necrotizing arteritis of PAN; destruction of vessel wall –> aneurysm

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7
Q

What does this image show?

A

Extravasation of blood into surrounding tissue in PAN

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8
Q

PAN clinical manifestations

A
  • Constitution
    • Fatigue
    • Fever
    • Weight loss
  • GI: abdominal pain
  • Kidney: HTN (2/2 renal artery occlusion; NO glomerulonephritis)
  • PNS: mononeuritis multiplex
  • Skin: nodules/ulcers, livedo reticularis (mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin)
  • Digital gangrene
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9
Q

What is livedo reticularis?

A

Enhanced prominence of the dermal or subcutaneous venous plexus due either to venodilatation and/or increased deoxygenation of the venous blood caused by regional ischemia secondary to reduced arteriolar blood flow.

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10
Q

What does this image show?

A

Livedo reticularis

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11
Q

PAN treatment

A
  • Onset:
    • Prednisone
    • Cyclophosphamide
    • Continue for at least one year
  • If HBV+:
    • Antiviral therapy (lamivudine)
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12
Q

What is kawasaki disease (mucocutaneous lymph node syndrome)?

A

Acute, febrile, multisystem disease of children characterized by nonsuppurative cervical adenitis and changes in the skin and mucous membranes such as edema; congested conjunctivae; erythema of the oral cavity, lips, and palms; and desquamation of the skin of the fingertips.

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13
Q

What are the clinical criteria for KD?

A
  • Fever for more than five days, plus:
  • 4 of 5 clinical criteria:
    • B/l nonpurulent bulbar conjuctival injection (spares limbus)
    • Oral mucous membrane inflammation
      • Injected and/or fissured lips
      • Nonpurulent erythematous pharynx
      • Strawbery tongue
    • Peripheral extremity abnormalities
      • Edema of hands and feet (acute)
      • Erythema of palms and soles
      • Periungual (surounding nail) desquamation (convalescent)
    • Polymorphous rash
    • Cervical LAD (1 LN > 1.5cm)
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14
Q

What is the epidemiology of KD?

A
  • >6mos to <5yo
  • Population differences <5yo:
    • Japanese: 112/100K
    • Korean: 86.4/100K
    • US: 9-33/100K
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15
Q

What does this image show?

A

Sheet-like desquamation of distal extremities; begins in periungual region of hands and feat; occurs during convalescent (resolution) phase

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16
Q

What is the etiology of KD?

A
  • Infectious?
    • ↑freq in household contacts
    • seasonal distribution (winter & summer)
    • occasionally occurs in epidemics
17
Q

KD complications

A
  • Coronary artery aneurysm 2/2 vasculitis occurring >10 days after onset of illness
    • Resulting in:
      • MI
      • arrhythmias
    • Frequency:
      • Untx: 25-30%
      • Tx: 5% (transient coronary artery dilatation); <1% (giant aneurysm >8mm)
18
Q

What does this image show?

A

coronary arteritis in KD

  • Panarteritis (all three layers of artery affected)
  • Minimal evidence of fibrinoid necrosis despite aneurysm formation
19
Q

KD treatment

A
  • Intravenous immunoglobulin (IVIG)
  • Aspirin
20
Q

What is the mechanism of activation of IVIG in KD?

A
  • ↓cytokines
  • neutralize bacterial superangtigens
  • augment T-cell suppressor activity
  • modulate endothelial cell function
  • reduce production of NO by neutrophils
  • Fc receptor blockade
  • anti-idiotypic inhibition of endothelial Abs
21
Q

What is giant cell arteritis (temporal arteritis)?

A

Inflammation of medium- and large-sized arteries, characteristically involving one or more branches of the carotid artery, particularly the temporal artery

22
Q

What vasculature is involved in GCA?

A

Above the diaphragm!

  • Thoracic aorta and major branches:
    • Carotid artery extra-cranial branches:
      • Temporal a.
      • Occipital a.
      • Facial a.
      • Ophthalmic a.
      • Posterior ciliary a.
    • Subclavian/axillary artery
23
Q

GCA epidemiology

A
  • >50yo (loss of inhibitory funct. of immune mech?)
  • Racial/ethnic background annual incidence:
    • 20/100K N. Europe
    • 2/100K AfAm & Hispanics
    • <1/1,000K Asians
24
Q

What does this image show?

A

Giant cell arteritis

25
Q

GCA clinical manifestations

A
  • Constitutional
    • Fatigue
    • Weight loss
    • Fever
  • H/A (66%): commonly temporal, but frontal or occiptal possible
  • Jaw pain (claudication: more pain with activity) (50%)
  • Visual loss (2/2 involvement of optic nerve)
    • Acute onset
    • Partial/complete visual field loss in 15-20% pts
  • Arm claudication (2/2 involvement of subclavian & axillary aa.) (15%)
26
Q

GCA pathogenesis

A
  • Unknown initial factor (perhaps a viral or other infection) activates dendritic cells located in the adventitia in medium and large blood vessel walls, initiating an adaptive immune response
  • TLR ligands recruit CD4 T cells which invade deeply into wall, causing panarteritis, whereas dendritic cells support perivascular infiltrates
  • Recruited cells cause further inflammation, tissue destruction and induction of repair mechanisms
  • Giant cells release vascular endothelial growth factor into intima, leading to luminal stenosis
27
Q

GCA laboratory abnormalities

A
  • ↑acute phase reactants
    • ESR
    • CRP
    • IL-6
28
Q

GCA diagnosis

A
  • Temporal artery bx
  • ↑ESR & CRP
29
Q

GCA treatment

A
  • **prednisone **tapering over 4-6mos
  • >30% relapse at dose of <15mg/day
  • in elderly, steroids assoc’d w/ ↑risk mortality, thus remove ASAP or use steroid-sparing agents
30
Q

What is polymyalgia rheumatica?

A

Inflammatory rheumatic condition characterized by aching and morning stiffness in the shoulders, hip girdle, and neck; can be associated with GCA

31
Q

PMR clinical manifestations

A
  • Intense pain and stiffness of musculature surrounding shoulders and pelvic girdle, typically b/l
    • Muscle strength normal
  • ​Fatigue & weight loss
  • ↑ESR or CRP
32
Q

PMR epidemiology

A
  • >50yo
  • Incidence at >50yo:
    • 113/100K N. Europeans
      • 2-3x more common than GCA
    • <2/100K Japanese
33
Q

What is the connection between PMR and GCA?

A
  • 10% of pts dx w/ PMR eventually develop features of GCA
  • 20-30% of pts dx w/ GCA eventually develop features of PMR

NB: monitoring for the two important b/c corticosteroid dosage differs

34
Q

PMR treatment

A
  • prednisone
    • prompt & dramatic response w/in 24-72hrs
35
Q

What is takayasu arteritis (pulseless disease)?

A

Inflammatory and stenotic disease of medium- and large-sized arteries characterized by a strong predilection for the aortic arch and its branches

36
Q

What vasculature is involved in TA?

A

Above and below the diaphragm

  • Thoracic aorta
    • Common carotid a.
    • Vertebral a.
    • Subclavian a.
  • Abdominal aorta
    • Celial a.
    • SMA
    • Renal a.
    • Iliac a.
  • Pulmonary artery
37
Q

TA epidemiology

A
  • Annual incidence 1.2-2.6/million
  • 15-25yo
  • F>M
  • ↑incidence Asians
38
Q

List the arteriographic abnormalities and their clinical symptoms in TA

A
  • Subclavian (93%): arm claudication, raynaud’s
  • Common carotid (58%): stroke, TIA, vision changes
  • Abdominal aorta (47%): abdominal pain
  • Renal (38%): HTN, renal failure
  • Aortic arch (35%): aortic insufficiency, CHF
  • Vertebral (35%): vision changes, dizziness
39
Q

TA treatment

A
  • prednisone
  • secondary immunosuppresive agents:
    • cyclophosphamide
    • methotrexate
    • azathioprine
    • infliximab

RMSK (16 decks)

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