Vasculitides Flashcards
Layers of vessels
Tunica Intima
Tunica Media
Tunica Adventitia
Typical symptoms
Organ ischemia (via thrombosis and inflammation- healing/ fibrosis)
Large vessel vasculitides
Affects Aorta/ Carotid
Giant Cell (Temporal)- most common (seen in females > 50) Takayasu (Asian females < 50)
Giant cell arteritis
Seen in females >50
Generally affects branches of carotid artery
Headache (temporal))
Visual disturbance
Jaw claudication
Polymyalgia rheumatica (flue like symptoms with myalgia)
Elevated ESR
Granulomatous vasculitis- inflamed vessel wall with giant cell and intimal fibrosis
Biopsy of giant cell
Need to take a large sample, because it is “segmental” in the way it affects
Negative biopsy doesn’t exclude giant cell arteritis, because it may be affecting a different area
Giant cell
Large cells with multiple nuclei
Treatment for giant cell arteritis
Corticosteroids- immediately when suspected
Because of high risk of irreversible blindness
Takayasu Arteritis
Similar to giant cell with a few exceptions:
- Adults <50 (typically young asian females)
- Affects aortic arch branch points (rather than more distal carotid artery)
Takayasa arteritis- treatment
Also corticosteroids
think of Takayasu as part of the spectrum of giant cell arteritis
Medium-vessel vasculitis
Affects muscular arteries (e.g. renal)
Polyarteritis Nodosa
Kawasaki
Buerger
Polyarteritis Nodosa
Necrotizing vasculitis
Involves most organs (but not lung)
Presents in young adults (renal- HTN, mesenteric- abdominal pain, neuro probs, skin probs)
Generally HBsAg +
Polyarteritis nodosa- histology
String of pearls appearance on imaging
Typically see transmural inflammation with fibrinoid necrosis (very pink on histology)- seen in vasculitis and malignant HTN
“Nodosa” because it appears like nodes after fibrosis occurs
Lesions are in different stages of healing (aneurysm- early lesions, fibrosis- late lesions)
Polyarteritis nodosa- tx
Corticosteroids or cyclophosphamide
Fatal if not treated
Kawasaki
Affects Asian children < 4
Looks like infection (fever, conjunctivitis, erythema in palms and soles, enlarged cervical lymph nodes)
Preferentially affects coronary arteries (can cause thrombosis and aneurysm)
Kawasaki- Tx
Aspirin- prevent thrombus formation in coronary arteries (important to distinguish that they do not have a viral illness)
IVIG
Disease is self limited
Buerger
Necrotizing- ulcerations and autoamputation
High association with smoking
Raynaud phenomenon seen in these patients
Small vessel vasculitis
Affects arterioles, capillaries, and venules
Wegener granulomatosis
Microscopic polyangiitis
Churg-Strauss
Wegener Granulomatosis “C”
Affects middle-aged mem
Involves nasopharynx, lungs, and kidneys
Patients present with rapidly progressing glomerulonephritis and hemoptysis
Necrotizing granulomas
c-ANCA positive
Cyclophosphamide & corticosteroids is the tx
Microscopic Polyangiitis
Similar to Wegner, but do not see:
No nasopharyngeal involvement (only affects lung and kidneys)
No granulomas seen
See p-ANCA not c-ANCA
Tx is the same as Wegner (cyclophosphamide and corticosteroids)
Churg-Strauss
p-ANCA +
Necrotixing granulomatous vasculitis with EOSINOPHILS
Typically have hx of asthma
Affects multiple organs: lungs and heart
Henoch Schonlein Purpura
Most common vasculitis in kids
PALPABLE purpura on buttocks and leg (because of inflammation)
GI pain and bleeding
Hematuria (IgA nephropathy)
Usually occurs following an upper respiratory tract infection- because of excess production of IgA
Generally self-limited
