16 Vasculitis Flashcards
Giant Cell (Temporal) arteritis affects what group most commonly?
Over 50. Especially Northern Europeans.
Giant Cell (Temporal) arteritis affects which arteries? (4)
temporal, vertebral, ophthalmic, aorta[group as mostly “arteries of the head”]
T/F temporal arteritis can cause permanent blindness?
T. (ophthalmic artery)
Suspected etiology/pathogenesis temporal arteritis?
Unknown. Suspected: immune rxn against arterial wall protein, probably T-cell mediated (granulomatous)
Symptoms of temporal arteritis?
fever, fatigue, weight loss, abrupt diplopia or vision loss[note most are non-specific]
Treatment temporal arteritis?
high-does anti-inflammatories (steroids)
Risk factors: Takayasu arteritis? (2)
-female under 40 (8:1, f:m)-HLA -A24, B52, DR2
Incidence: Takayasu arteritis? (2)
-US: 1/1000-Asia: 6/1000
Giant Cell (Takayasu) arteritis: affects what body parts? (3) How?
-Ascending aorta “Pulseless disease”-Can also involve pulmonary & renal arteries-Fibrosis of artery wall ->stenosis-> occlusion->dilation-> aneurysm
Giant cell (Takaysu) arteritis: signs early & late phases?
-Early: fever, arthalgias, weight loss, “prepulseless”-Late: “Pulseless”
Type 1 Takayasu Arteritis is?
branchiocephalic trunk, carotid arteries & subclavian
Type 2 Takayasu Arteritis is?
combined Type 1 & Type 3
Type 3 Takayasu Arteritis is?
“atypical coarctation type” involves thoracic & abdominal aorta (most common!!)
Type 4 Takayasu Arteritis is?
“Dilated type”, dilation of aorta & its major branches
Takayasu arteritis: treatment? (2)
-steroids-angioplasty (after acute phase)
Thromboangitis obliterans (Buerger’s disease): Risk factors? (3)
-Japan or Near East descent-Smoking-HLA-A9, HLA-B5
Thromboangitis obliterans: etiology, pathogenesis? (3)
-Endothelial damage-Hypersensitivity to tobacco extract-Result: acute & chronic vasculitis of medium & small arteries, esp radial & tibial. -> Ulceration of fingers & toes
Thromboangitis obliterans: treatment?
Quit smoking!
Wegener’s granulomatosis (Granulomatosis with polyangitis): risk factors?
-Any age, sex, race, but mostly whites w/ slightly more males-Age 41 median onset
Wegener’s granulomatosis (Granulomatosis with polyangitis): etiology, pathogenesis? (4)
- Unknown. Suspected: Hypersensitivity rxn initially
- Necrotizing granulomatous vasculitis
- Focal segmental glomerular nephritis
- Necrotizing vasculitis of median & small vessels
Kawasaki’s/Mucocutaneous Lymph Node Syndrome: risk factors? (2)
- Under 4 yrs
- USA or Japan [#1 ACQUIRED hearts disease in these countries]
Kawasaki’s/Mucocutaneous Lymph Node Syndrome: pathogenesis?
-anti-endothelial, anti-smooth muscle Ab’s, and T-cell attack ->coronary artery vasculitis-> Possible MI, rupture, ectasia, or thrombosis.
Kawasaki’s/Mucocutaneous Lymph Node Syndrome: etiology
possibly viral infection
Kawasaki’s/Mucocutaneous Lymph Node Syndrome: symptoms?
fever, rash, swollen hands/feet w/ desquamation, red eyes, swollen neck lymph nodes, strawberry tongue/mouth/throat
Kawasaki’s/Mucocutaneous Lymph Node Syndrome: treatment?
aspirin, IVIG
Polyarteritis Nodosa: What does it affect? (3)
- small-medium vessels
- May involve any organ. Commonly skin, joints, peripheral nerves, GI, kidney
- Often with Hepatitis B
Polyarteritis Nodosa: pathogenesis? (4)
- panmural necrotizing arteritis with fibrinoid necrosis! esp. at branch pt (either circumferential or segmental)
- mixed cell infiltrate
- aneurysmal dilatation
- heal w/ fibrosis
Polyarteritis Nodosa: diagnosis? (2)
- angiography or histology
- ANCA 80% positive, equally distributed c and p forms
Polyarteritis Nodosa: treatment/prognosis?
- glucocorticoids
- half die in 5 yrs, most in first year
which CAN have granulomas: polyarteritis nodosa or microscopic polyangitis?
Poly nodosa
which has medium and small vessels: polyarteritis nodosa or microscopic polyangitis?
poly nodosa (MICROSCOPIC polyangitis has only SMALL)
Which has glomerulonephritis: polyarteritis nodosa or microscopic polyangitis?
microscopic polyangitis
Which has pulmonary hemorrhage: polyarteritis nodosa or microscopic polyangitis?
microscopic polyangitis
Which has relapses frequently? polyarteritis nodosa or microscopic polyangitis?
microscopic polyangitis
Which has abnormal angiography: polyarteritis nodosa or microscopic polyangitis?
poly nodosa
Which has renal vasculitis w/ renal hypertension & infarcts: polyarteritis nodosa or microscopic polyangitis?
poly nodosa
[note: micro polyangitis had glomerulonephritis!]
Wegener’s granulomatosis (Granulomatosis with polyangitis): manifestations? (3)
- sinusitis, nasal obstruction, otitis media, hearing loss, skin rash, fever, arthralgia, weight loss
- lung involvement: 45% at presentation ->85% in all. Pulm nodules, hemoptosis, pleuritis
- 80% glomerulonephritis
Wegener’s granulomatosis (Granulomatosis with polyangitis): diagnosis? (5)
- Lung biopsy
- Vessels w/ fibrinoid necrosis + PMN’s
- renal biopsy
- gingival biopsy
- ANCA: 90% c and 10% p
Wegener’s granulomatosis (Granulomatosis with polyangitis): treatment & prognosis? (2)
- cyclophosphamide (chemotherapy)
- remission partial in 90%, full in 75%, 1/2 relapse in 3 yrs
Eosinophilic granulomatosis with polyangiitis a.k.a. allergic angitis a.k.a. granulomatosis a.k.a. Churg Strauss: risk factors? (1)
-25-69 yrs, median 38-50
Eosinophilic granulomatosis with polyangiitis a.k.a. allergic angitis a.k.a. granulomatosis a.k.a. Churg Strauss: pathogenesis? (3)
- vascular & extravascular granulomatous inflammation
- allergic rhinitis, severe asthma, multiple organs
- hypereosinophilia
Eosinophilic granulomatosis with polyangiitis a.k.a. allergic angitis a.k.a. granulomatosis a.k.a. Churg Strauss: clinical course? (3)
Phases:
- Prodromal: allergic respiratory disease
- Eosinophilic: peripheral blood & tissue eosinophilia
- Vasculitic
Eosinophilic granulomatosis with polyangiitis a.k.a. allergic angitis a.k.a. granulomatosis a.k.a. Churg Strauss: diagnosis?
-ANCA 70-80% positive, usually P & anti-MPO
Behcet’s Disease: risk factors?
-middle east, japan, china “silk route”
Behcet’s Disease: Manifestations? (6)
- All artery types
- aphthous ulcers in mouth
- pustular lesions on skin & scarring erythema nodosum
- scrotal, vulvar ulcers
- uveitis
- lung aneurysms
Behcet’s Disease: diagnosis?
pathergy test (sterile pin evokes allergic rxn!)
Behcet’s Disease: treatment? (2)
immunosuppression, thalidomide
Primary CNS Vasculitis: what’s the only type that gets better over time?
BACNS (benign angiopathy of the CNS)
Primary CNS Vasculitis: risk factors?
4:3 female to male. BACNS typically is in young women.
Primary CNS Vasculitis: symptoms? (8)
- headache
- organic brain syndrome
- multifocal neurologic deficits
- seizures
- strokes
- confusion
- memory loss
- altered consciousness
Primary CNS Vasculitis: etiology?
maybe coffee, nicotine, or contraceptives, unknown really.
Primary CNS Vasculitis: diagnosis?
- angiography or MRI (still not certain)
- Brain and meningeal biopsy (25% false negatives still)
Primary CNS Vasculitis: treatment?
massive immunosuppression. Usually fatal within 45 days.
BCANS (benign angiopathy of the CNS) subset of CNS vasculitis: treatment?
Ca channel blockers, short course steroids, get better!
Raynaud’s phenomena classic risk factors?
woman in cold climate w/ emotional stress, age 20-40, with a cigarette in one hand, a bottle of alcohol in the other hand, and munching on an estrogen replacement pill in her mouth.
Raynaud’s phenomena color change?
white, blue, red
[no blood, vessels dilate to keep blood, flow returns]
Raynaud’s phenomena has what causes? (2)
- Primary, less severe & bilateral
- Secondary: not bilateral, associated w/ connective tissue disorder
Raynaud’s phenomena treatment?
eliminate the risk factors, and give sympathetic nerve blockers
Raynaud Syndrome etiology & pathogenesis?
cryoglobulins occlude vessels @ low temp ->purpuric lesions
Raynaud Syndrome risk factors? (3)
- collagen vascular diseases (SLE, R.A., Scleroderma)
- infectious disease
- hematologic disorders
Raynaud Syndrome treatment?
keep warm, treat underlying disease
