Vasculitides
1. Classification of the vasculitides based on a. The size of the blood vessel involved b. Current knowledge of disease pathophysiology c. The pattern of organ involvement 2. Subclassification of Vasculitides a. Based on certain pathophysiologic features
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Section A: Polyarteritis Nodosa, Microscopic Polyangiitis and Small-Vessel Vasculitides 1. Classification of the vasculitides based on a. The size of the blood vessel involved b. Current knowledge of disease pathophysiology c. The pattern of organ involvement 2. Subclassification of Vasculitides a. Based on certain pathophysiologic features b. Immune complex mediated c. Association with characteristic autoantibodies, i. Such as antineutrophil cytoplasmic antibodies (ANCA) d. The presence or absence of granulomatous inflammation e. Trophism of particular organs i. Microscopic polyangiitis (MPA) and cutaneous leukocytoclastic angiitis (CLA) both are similar clinically and pathologically 1. Only CLA is confined to the skin 3. Characteristics of Small Vessel Vasculitides a. More numerous b. Affect blood vessels that are lower) c. Synovitis iii. GCA as Fever of Unknown Origin (FUO) (15%) 1. Fever and systemic symptoms without any localized symptoms 2. Spiking temperatures and chills prompt a FUO work-up 3. Symptoms a. Low-grade fever b. Malaise c. Anorexia d. Weight loss e. Fatigue 4. Symptoms of vascular insufficiency can be absent 5. Physical exam of the scalp arteries often negative iv. Large-vessel variant GCA 1. Large arteries targeted in 10–15% 2. Arteries most frequently involved a. Carotid b. Subclavian c. Axillary arteries 3. Arteries less frequently involved a. Femoral arteries 4. Lack evidence of cranial involvement a. Do not complain of headaches 5. Temporal arteries a. Appear normal on exam b. Almost 50% of biopsies are negative for vasculitis 6. Angiography a. Diagnostic procedure of choice 7. PET scan a. May suggest aortic involvement b. Even if not detected clinically 8. Stenotic vascular lesions a. Typically located at the subclavian axillary junction b. Unilateral 9. Aortic arch syndrome a. The major clinical presentation b. Claudication of the arms c. Absent or asymmetrical pulses d. Paresthesias e. Raynaud’s phenomenon
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Vasculitides
f. Peripheral gangrene g. Aortitis can coexist with cranial arteritis 10. Thoracic aortic aneurysm a. A 17 fold increased risk b. Elastic membranes destroyed i. Replaced by fibrotic tissue c. Clinical spectrum i. Silent aneurysm ii. Aortic dissection iii. Fatal rupture g. Laboratory features i. A pathognomonic laboratory test does not exist ii. Acute phase reactants 1. Typically highly elevated iii. ESR 1. The most useful laboratory test 2. A sensitive indicator of GCA 3. Specificity 50 mm/h b. Averages 80–100 mm/h (Westergren method) 5. May be normal (rare) a. Associated with corticosteroid treatment for other conditions (polymyalgia rheumatica (PMR)) iv. CRP 1. Elevated levels may be more sensitive for the acute phase response 2. Frequently at high levels (>10 mg/dl) v. IL-6 (serum) 1. Marker with the highest sensitivity for detecting ongoing systemic inflammation 2. Taken before and after corticosteroid therapy 3. A strong inducer of acute phase reactants vi. Anemia 1. Mild to moderate 2. Normochromic or hypochromic vii. Platelet counts 1. Commonly elevated
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