Vasculitis - Langford.pdf - AInotes

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Urticarial Vasculitis Laboratory Studies and Investigations Obtain in patients with a compatible clinical and histologic picture to detect hypocomplementemia and systemic features: • Complement (CH50, C3, C4, C1q) • CBC with differential • Urinalysis • Chemistries • Hepatitis B and C serologies • ANA, anti-DNA, ENA • ANCA • RF and anti-CCP • SPEP • Cryoglobulins Consider other investigations as appropriate: • chest imaging • Skeletal radiographs in patients with joint pain • PFTs (to look for COPD)
Urticarial Vasculitis Hypocomplementemic Urticarial Vasculitis Syndrome (HUVS) Specific autoimmune disorder that involves > 6 months of urticaria with hypocomplementenia in the presence of systemic findings Clinical features • Urticarial vasculitis is the dominant feature • Angioedema occurs in 50% and can be the presenting feature • Moderate to severe COPD occurs in 50% • Ocular inflammation (uveitis( uveitis) ) occurs in 30% • Can get glomerulonephritis • Many features resemble SLE (some propose it is a subset of SLE) Labs • C3, C4 can be undetectable to low normal • C1q low in all patients when disease is active • Anti-C1q antibodies (C1q precipitins) detectable in all patients • Anti-DNA and anti-Sm are uncommon
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Vasculitis Carol A. Langford, MD, M
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CME Disclosure Statements Carol A.
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Vasculitis = Inflammation of the Bl
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Example Clinical Consequence Vein V
Page 9 and 10: Vein Venule Capillary Arteriole Art
Page 11 and 12: Giant Cell Arteritis (Also called t
Page 13 and 14: Giant Cell Arteritis Diagnosis Sugg
Page 15 and 16: Kawasaki Disease Diagnosis Epidemio
Page 17 and 18: Kawasaki Disease Primary complicati
Page 19 and 20: Kawasaki Disease Treatment Outcome
Page 21 and 22: Polyarteritis Nodosa Often presents
Page 23 and 24: Polyarteritis Nodosa Diagnosis Arte
Page 25 and 26: Daily Cyclophosphamide A life savin
Page 27 and 28: Wegener’s Granulomatosis Clinical
Page 29 and 30: Wegener’s Granulomatosis Clinical
Page 31 and 32: Antineutrophil Cytoplasmic Antibodi
Page 33 and 34: Methods of ANCA Testing Indirect Im
Page 35 and 36: Key Issues Regarding ANCA Clinical
Page 37 and 38: Wegener’s Granulomatosis Treatmen
Page 39 and 40: Microscopic Polyangiitis Clinical M
Page 41 and 42: Microscopic Polyangiitis Treatment
Page 43 and 44: Churg-Strauss Syndrome Clinical Man
Page 45 and 46: Churg-Strauss Syndrome Treatment Ou
Page 47 and 48: Henoch-Sch Schönlein Purpura 4 car
Page 49 and 50: Henoch-Sch Schönlein Purpura Treat
Page 51 and 52: Cryoglobulinemic Vasculitis Clinica
Page 53 and 54: Cryoglobulinemic Vasculitis Diagnos
Page 55 and 56: Cutaneous Leukocytoclastic Vasculit
Page 57 and 58: Urticarial Vasculitis Consists of t
Page 59: Urticarial Vasculitis Normocompleme
Page 63 and 64: Urticarial Vasculitis Treatment Tre
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Vasculitis - Langford.pdf - AInotes

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