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39 Cards in this Set
- Front
- Back
3 mechanisms of Vascultits |
1. pathogenic immune complex formation/ deposition 2. production of ANCA 3. Pathogenic T lymph responses & granuloma formation |
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What diseases cause pathogenic immune complex formation/deposition vasculitis? |
Henoch-Schonlein purpura Vasc assoc w/ CVD Serum sickness & cutaneous vasc syndromes Hep C (mixed cryoglobinemia) Hep B (Polyarteritis nodosa) |
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What diseases cause production of ANCA leading to vasculitis? |
Wegner's Granulomatosis Churg Strauss syndrome microscopic polyangiitis |
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what diseases cause pathogenic T lymph responses & granuloma formation leading to vasculitis? |
Giant Cell arteritis Takayasu's arteritis Wegners Granulomatosis Churg Strauss syndrome |
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Vasculitis in inflammation of blood vessels that leads to ...... |
narrowing of the lumen
(due to thickening of the walls)
(may eventually lead to blockage, aneurysm, or rupture) |
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What pathology is this? What do the purple dots represent? |
Panarteritis (Vasculitis)
purple dots= inflammatory cells w/i the vessel |
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Constitutional symptoms of Vasculitis: |
fever weight loss malaise arthralgias/arthritis |
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Symptoms associated w/ large vessel vasculitis |
Limb claudication (cramping) Asymmetric BP Absence of pulses Bruits Aortic dilation |
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Large & medium vessel vasculitis |
Takayasu's Arteritis (pulseless) Giant cell arteritis (Temporal) |
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Symptoms associated w/ medium vessel vasculitis |
cutaneous nodules ulcers livedo reticularis digitial gangrene mononeuritis multiplex microaneurysma
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medium & small vessel vasculitis |
kawasaki disease Polyarteritis nodosa (PAN) Wegners granulomatosis Churg-Strauss syndrome (allergic granuloma & angitis)
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Symptoms associated w/ small vessel vasculitis |
purpura vesiculobullous lesions urticaria glomulonephritis alveolar hemorrhage cutaneous extra-vascular necrotizing granulomas splinter hemorrhages uveitis episcleritis scleritis |
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Small vessel vasculitis |
necrotizing glomerulonephritis microscopic polyangitis Henoch-Schonlein purpa Hypersensitivity Vasulitis Cryoglobinemia |
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What lab tests indicate vasculitis? |
anemia high LFT (liver enzymes inc) hematuria high ESR (segmentation rate = inflammation) |
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What is wrong w this finger? |
purpuric lesion (or infarct)
= small vessel vasculitis |
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What is wrong in this picture? |
retinal vessels leaking exudates
=retinal vasculitis |
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What is wrong in this pic? |
scleritis & episcleritis |
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What is wrong in this image? |
cavitary pulmonary lesion w/ inflammation |
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What pathology? What is the sample of? What is the yellow portion (red bracket)? |
Cryoglobulinemia (causing purpura on skin)
-serum sample w/ yellow cryocrit
(must take blood & keep at body temp until serum extracted, then refridgerate to seperate cryocrit (immune complex deposits)) |
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Palpable purupra occurs in what conditions? |
-serum sickness -Henoch-schoenlein purpura -essential mixed cryoglobinemia |
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Palpable purpura is clinically classified as ___________________
What causes the painful purple sores? |
Small vessel vasculitis/ Leukocytoclastic vasculitis (LVC)
-skin w/ PMNs & mononuclears cells leading to fibrinoid necrosis & wbc fragments (= purpura) |
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Which disease? -typical in children -seasonal (spring) -immune-complex depos. of IgA -Arthritis -Nephropathy -GI (bleeding, abdominal pain) -Purpura -small vessel vasculitis |
Henoch-Schoenlein Purpura |
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What disease? -mixed immun-complex depos. of IgG & IgA (cold precip. complexes) -often have a + rheumatoid factor (w/o RF) -polyarthralgia -Nephritis -Peripheral neuropathy -Assoc. w. Hep C -Lower extremity purpura -small vessel vasculitis
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Essential Mixed Cryoglobinemia
(essential if in absense of another disease) |
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In addition to purpura, what are the other clinical symptoms of cryoglobulinemia? |
-Raynaud's -Cutaneous ulcerations (esp distal extremities) -Cold-induced urticaria (rash from cold) -Digital gangrene |
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What disease? -vascular lesions -nectrotizing inflammation of muscular arteries -involved artery bifurcations & branching -Hep B antigenemia -small & medium vessel vasculitis
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Polyarteritis Nodosa (PAN) |
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ACR classification criteria for polyarteritis nodosa |
-testicular pain (middle age man)* -myalgias, weakness, or leg tenderness -weight loss > 4 kg -livedo reticularis -mono/poly-neuropathy -diastolic BP > 90 -azotemia -Hep B -arteriographic abnormality (aneurysm) -biopsy containing PAN
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What is wrong w this image? What pathology? |
-giant aneurysm -arteries look like stringed sausage (not straight)
Polyarteritis Nodosa (PAN) |
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What disease? (ACR Diagnosis MUST include 4/6 below) 1. Mono/poly-neuropathy 2. Eosinophils (in tissue) 3. Asthma 4. Tranistory pulmonary infiltrates 5. paranasal sinus abnormalitis 6. biopsy w/ extravascular eosinophils
-granulomatous rxn -small & medium vessel vasculitis |
Churg-Strauss syndrome (allergic angiitis & granulomatosis) |
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What disease? (ACR Dx MUST include 2/4) 1. Nasal or Oral ulcers or bloody nasal drainage 2. Abnormal chest X-ray (nodules, infiltrates, cavities) 3. Urinary sediment (>5 RBC or casts) 4. granulomatous vasculitis on biopsy
-glomerulonephritis -ELK classification -small & medium vessel vasculitis |
Wegner's granulomatous |
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Wegner's granulomatous usually occurs in middle-aged (NOT black) individuals w/ cough & hypertension. What Abs are associated w/ Wegners? |
C-ANCA (anti PR-3)
(^Ab directed against neutrophils & monocytes)
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What disease? |
Wegner's Granulomatosis
(rounded granulomas on histology & intense infiltrates & cavities on x-ray) |
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What disease? (ACR Dx need 3/6) 1. Age < 40 (esp young/adolescent women) 2. claudication (cramping) of extremities 3. dec brachial artery pulse 4, BP diff > 10 btwn arms 5. Bruits over subclavian or Aorta 6. Arteriogram abnorm. (narrowing/occlusion aortic arch & branches)
-more common in Asia -medium & large vaculitis |
Takayasus Arteritis (pulseless) |
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What are the arrows pointing at? What disease? |
narrowing near aortic arch
Takayasu's Arteritis (pulseless disease) |
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What disease? (ACR Dx need 3/5) 1. Age > 50 (at onset) (old) 2. New Cephalgia (headache) 3. Temporal Artery abn. (tender, dec pulse, risen) 4. elevated ESR > 50mm/hr (sediment rate) 5. Abn. biopsy (mono infiltrate, granulomatous inflam, usually multinuc giant cells)
-usually involves carotid artery -scandinavian women likely -jaw claudication or muscle pain -medium & large vasculitis |
Giant Cell (Temporal Arteritis) |
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____________ is a vasculitis w/ subcutaneous fat inflammation
associated w; -Erythema nodosum -Weber-Christian Disease -Pancreatis |
Panniculitis |
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What is the pathology? What are the white dots? |
Panniculitis
-fatty nodules under skin (may push up on skin & cause pain) (purple septal areas are inflammatory cells) |
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What does this pt have on ant lower legs? |
erythema nodosum (assoc w/ panniculits, also assoc. w/ sarcoidosis, strep, Tb) |
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How are vaculitides treated? |
-glucocotricoids & immunosuppressives -cyclophosphamide for severe forms |
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Which vasculitis involve veins (as well as arteries)? |
Wegner's Granulomatosis
(H-S purpura & hypersensitivities may involve venules)
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