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27 Cards in this Set

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Vasculitis: definition
Inflammation and necrosis of blood vessels:
Dilation/rupture
Thrombosis/ischemia
Distinct from vasculopathy
Vasculitis: Classification
Large vessel: aorta and branches (Giant cell arteritis)
Medium vessel: muscular arteries, arterioles (classic polyarteritis nodosa)
Small vessel: capillaries, venules (Wegener's granulomatosis)
Vasculitis:Constitutional symptoms
Fever, weight loss, anorexia, fatigue
Vasculitis: Small vessel skin manifestations
Palpable pupura, urticaria, non-palpable purpura, vesicles/bullae, splinter hemorrhage, superficial ulceration
Vasculitis: Medium vessel skin manifestations
Livedo reticularis, nodules, ulcerations, infarcts, gangrene
Vasculitis: Joint/muscle manifestations
Arthralgias, arthritis, myalgias+/-weakness
Vasculitis: Kidney manifestations
Focal necrotizing glomerulonephritis: hematuria, proteinuria, renal failure, hypertension
Vascular nephropathy (infarcts)
Vasculitis: Nervous system manifestations
Peripheral nerve: mooneuritis multiplex (emergency), polyneruopathy
CNS: stroke, seizure
Vasculitis: Heart/lungs manifestations
Congestive heart failure, myocardial infarction, pericarditis
Pulmonary infiltrates/nodules, pulmonary hemorrhage
Vasculitis: GI manifestations
Bowel ischemia/infarct
Vasculitis: ENT/eye manifestations
Episcleritis, orbital pseudotumor, chronic sinusitis, saddle nose deformity
Vasculitis: other manifestations
Arm/leg claudication
Jaw claudication
Scalp tenderness
Testicular pain
Vasculitis: Diagnosis
Bloodwork: Inflammatory markers, organ systems, immune complexes, antibodies (ANCA)
Tissue biopsy
Imaging
Giant Cell Arteritis: Epidemiology
Most frequent vasculitis in N.A.
>50yo
F>M
North to South gradient
Giant Cell Arteritis: symptoms and signs
constitutional symptoms, new onset headache, jaw claudication, shoulder/pelvic pain/stiffness, plymyalgia rheumatica, diploplia, scalp tenderness
Giant Cell Arteritis: anterior ischemic optic neuropathy (AION)
End result of untreated giant cell arteritis
Permanent vision loss
*must do biopsy promptly to diagnose
Giant Cell Arteritis: treatment
Corticosteroids: 1mo high dose, taper, 1-2 years low dose
Giant Cell Arteritis: prognosis
Median survival: 6 years
Giant Cell Arteritis: complications
Thoracic aortic aneurysm
Aortic dissection
*do yearly CXR to monitor
Polyarthritis nodosa: epidemiology
M=W
40-60yo
Hepatitis B a risk factor
Rare associations: HIV, CMV, parvovirus B19, HCV
Polyarthritis nodosa: Signs and Symptoms
Weight loss>4kg
Livedo reticularis
Myalgias
Mononeuritis multiplex
Bowel ischemia
Renal infarcts
Wegener's granulomatosis: organ system's involved
Pulmonary (major), ENT, renal, skin, nerves
Wegener's granulomatosis: antineutrophil cytoplasmic antibody formation
1. Infection causes presentation of cytoplasmic proteins on neutrophil cell surface
2. Antibodies against protiens is made (ANCA)
3. ANCA stimulate neutrophils further
Wegener's granulomatosis: diagnosis
Tissue biopsies:
lungs (open biopsy best): necrosis, vasculitis, granulomas
renal: glomerulonephritis, necrosis, pauci-immune, vasculitis (rare), granulomas (rare)
Diseases that are positive for ANCA
Wegener's granulomatosis
Microscopic polyangitis
Glomerulonephritis
Wegener's granulomatosis: treatment
Corticosteroids
Immunosuppressive agents: cyclophosphamide, methotrexate, azathioprine, mycophenolate
Wegener's granulomatosis: prognosis
5 year survival = 80-90%