Vasculitis Flashcards
What is vasculitis?
Inflammation of the blood vessel wall
What are the three layers of the arterial wall?
Endothelial intima (inner most)
Smooth Muscle media
Connective tissue adventitia
What is the etiology of vasculitis?
Usually unknown, most cases are not infectious
What are the clinical features of vasculitis?
- Nonspecific symptoms of inflammation (e.g. fever, fatigue, weight loss, and myalgias)
- Symptoms of organ ischemia (due to luminal narrowing (from fibroblasts) or thrombosis (endothelial cells taken off and under part exposed - attracts platelets) of the inflamed vessels
What does large vessel vasculitis involve?
Aorta and its major branches
What does medium vessel vasculitis involve?
Muscular arteries that supply organs
What does small vessel vasculitis involve?
Arterioles, capillaries and venules
What groups is vasculitis divided into?
- Large-vessel
- Medium-vessel
- Small-vessel
What are two types of Large Vessel vasculitis?
- Temporal (Giant Cell) Arteritis
- Takaysu Arteritis
What is Temporal (Giant Cell) Arteritis?
Granulomatous vasculitis that classically involves branches of the carotid artery
What is the usual population associated with Temporal (Giant Cell) Arteritis?
Females
What is the most common form of vasculitis in older adults?
Older adults >50 yrs
Temporal (Giant Cell) Arteritis
What is the typical presentation of Temporal (Giant Cell) Arteritis?
- Headache (temporal artery involvement)
- Visual disturbances (ophthalmic artery involvement)
- Jaw claudication
- Flu-like symptoms
- Joint and muscle pain (polymyalgia rheumatica)
- ESR is elevated (often >100)
What does biopsy show in Temporal (Giant Cell) Arteritis?
Inflamed vessel wall (vasculitis) with giant cells and intimal fibrosis.
- Requires biopsy of a long segment of vessel
- A negative biopsy does not exclude disease
What are the features of lesions in Temporal (Giant Cell) Arteritis?
Lesions are segmental
How do you treat Temporal (Giant Cell) Arteritis?
Corticosteroids
-High risk of blindness without treatment so you need to treat ASAP
What is Takayasu Arteritis?
Granulomatous vasculitis that classically involves the aortic arch at branch points.
What is the usual population that presents with Takayasu Arteritis?
Adults
What symptoms does Takayasu Arteritis usually present with?
-Neurological and visual symptoms with a weak or absent pulse in the upper extremity (‘pulseless disease’)
What do labs show in Takayasu Arteritis?
Elevated ESR
What is the treatment of Takayasu Arteritis?
Corticosteroids
What are the three types of Medium Vessel Vasculitis?
- Polyarteritis Nodosa
- Kawasaki Disease
- Buerger Disease
What is Polyarteritis Nodosa?
Necrotizing vasculitis involving multiple organs
- Lungs are spared
- Involves “many arteries”
What symptoms does Polyarteritis Nodosa present with?
Usually presents in young adults as:
- Hypertension (renal artery involvement)
- Abdominal pain with melena (black ‘tarry’ feces) (mesenteric artery involvement)
- Neurologic disturbances
- Skin lesions
What does Medium-Vessel Vasculitis involve?
Muscular arteries that supply organs (ex: renal artery)
What lab is Polyarteritis Nodosa associated with?
Serum HBsAg
-Hep B surface antigens found in these patients
What types of lesions do you see with Polyarteritis Nodosa?
- Lesions of varying stages are present
- Early lesion consists of transmural inflammation with fibrinoid necrosis (highlighter pink)
- This fibrinoid necrosis eventually heals with fibrosis, producing a ‘string-of-pearls’ appearance on imaging
What do you see upon imaging of Polyarteritis Nodosa?
“String-of-pearls” appearance on imaging
-Caused by aneurysms
How do you treat Polyarteritis Nodosa?
-Corticosteroid
and
-Cyclophosphamide
What can happen if you do not treat Polyarteritis Nodosa?
It can become fatal.
