16: Medium & Large Vessel Vasculitis Flashcards
What are the types of medium and large vessel vasculites?
- Medium vessel:
- Polyarteritis nodosa
- Kawasaki disease
-
Large vessel:
-
Giant cell arteritis
- Polymyalgia rheumatica
- Takayasu’s arteritis
-
Giant cell arteritis
What is polyarteritis nodosa (PAN)?
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
PAN affected vasculature
Primarily below the diaphragm
- SMA
- Celiac & hepatic arteries
- Renal arteries
- Musclar arteries of extremities
- Coronary arteries (uncommon)
PAN epidemiology
- 20-70yo
- No racial/ethnic predilection
- Incidence:
- 2-4/1,000K annually in US
- 70-80/1,000K annually in regions endemic for HBV
What is the association between PAN and HBV?
PAN occurs acutely in first 6mos following HBV infx; usually + for surface and e antigens
What does this image show?
Necrotizing arteritis of PAN; destruction of vessel wall –> aneurysm
What does this image show?
Extravasation of blood into surrounding tissue in PAN
PAN clinical manifestations
- 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
What is livedo reticularis?
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.
What does this image show?
Livedo reticularis
PAN treatment
- Onset:
- Prednisone
- Cyclophosphamide
- Continue for at least one year
- If HBV+:
- Antiviral therapy (lamivudine)
What is kawasaki disease (mucocutaneous lymph node syndrome)?
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.
What are the clinical criteria for KD?
- 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)
What is the epidemiology of KD?
- >6mos to <5yo
- Population differences <5yo:
- Japanese: 112/100K
- Korean: 86.4/100K
- US: 9-33/100K
What does this image show?
Sheet-like desquamation of distal extremities; begins in periungual region of hands and feat; occurs during convalescent (resolution) phase
What is the etiology of KD?
- Infectious?
- ↑freq in household contacts
- seasonal distribution (winter & summer)
- occasionally occurs in epidemics
KD complications
- 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)
- Resulting in:
What does this image show?
coronary arteritis in KD
- Panarteritis (all three layers of artery affected)
- Minimal evidence of fibrinoid necrosis despite aneurysm formation
KD treatment
- Intravenous immunoglobulin (IVIG)
- Aspirin
What is the mechanism of activation of IVIG in KD?
- ↓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
What is giant cell arteritis (temporal arteritis)?
Inflammation of medium- and large-sized arteries, characteristically involving one or more branches of the carotid artery, particularly the temporal artery
What vasculature is involved in GCA?
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
- Carotid artery extra-cranial branches:
GCA epidemiology
- >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
What does this image show?
Giant cell arteritis
