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69 Cards in this Set
- Front
- Back
C-ANCA acts against
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PR3- proteinase 3 in neutrophils
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P-ANCA acts against
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MPO- myeloperoxidase
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C-ANCA indicative of
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Wegener Granulomatosis
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P-ANCA indicative of
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Microscopic Polyarteritis
Churg Strauss Syndrome |
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Fragmentation of internal elastic lamina
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Giant cell arteritis (Temporal arteritis)
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Clinical symptoms:
1- Fever, fatigue, weight loss 2- Pain or headache along course of a tender, nodular, temporal artery 3- Vision changes 4- Myocardial ischemia, GI, neurologic changes 5- Polymyalgia rheumatica |
Giant cell arteritis
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Difference between Takayasu disease and temporal arteritis
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1- YOUNGER <40 yrs old Japanese women
2- Aortic arch affected |
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Polyarteritis nodosa microscope
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Artery with segmental, transmural inflammation with fibrinoid necrosis
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Clinical Polyarteritis
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1) Skin lesions- purpura, livedo reticularis, Infarcts of tips of digits
2) Renal- HTN, failure 3) Peripheal neuropathy 4) Fever, weight loss, malaise 5) GI- Adbdominal pain, bleeding, pain 6 )Aneurysm 7) Hepatitis B 8) Testicular pain Vessels at different (ALL) stages of inflammation Remember doesn't affect lungs and NO glomerulonephritis!!! |
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Major cause of death of Polyarteritis Nodosa
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Renal involvement
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What does polyarteritis nodosa NOT affect?
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Lungs!
Arterioles, venules, capillaries |
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How is microscopic polyarteritis or polyangitis different from PAN? (4)
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1- Affects arterioles, capillaries, venules
2- Inflammatory lesions of the SAME stage 3- p-ANCA present in 80% of cases 4- Necrotizing glomerulonephritis and pulmonary capillaritis are common |
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Leukocytoclastic vasculitis lesions are
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Purpuras
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Churg-Strauss Syndrome features
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Age- 50 years, SMALL VESSELS
1- Allergic rhinitis 2- Asthma 3- Eosinophila 4- P-ANCA |
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Triad features in Wegener granulomatosis
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1- Necrotizing granulomatous vasculitis
2- Necrotizing granulomata of lungs and upper airways 3- Glomerulonephritis |
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c ANCA and Wegener granulomatosis
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C-ANCA is positive in 90% of cases
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Wegener's granulomatosis
What is it? Symptoms Findings Treatment |
Triad of:
1- Focal necrotizing vasculitis 2- Necrotizing granulomas in the lung and upper airway 3- Necrotizing glomerulonephritis -Affects medium and small vessels Symptoms- hemoptysis, hematuria, perforation of nasal septum Findings- C-ANCA is strong marker |
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Buerger's Disease (Thromboangiitis obliterans)
Pathogenesis Clinical features |
Pathogenesis- Cigarette smoking causes endothelial cell damage due to direct cytotoxicity or hypersensitivity
Clinical 1) Raynaud 2) Pain in instep area 3) Chronic ulcers, gangrene of digits, feet |
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What happens in Raynaud's Phenomenon?
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White, blue, and red response
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Primary Raynaud's Phenomenon is caused by
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Unknown
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Secondary Raynaud's Phenomenon is caused by
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Arterial insufficiency due to diseases such as SLE, SS, AS, or Buerger's Disease
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Difference between juvenile vs cavernous hemangiomas (port-wine stains)
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Cavernous hemangiomas do not regress
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Pyogenic granuloma
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Occurs after trauma in skin, gingival, or oral mucosa
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Cystic hygroma is what
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Cavernous lymphangioma, occurs in neck or axilla
-Associated with Turner's Syndrome |
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One major cause of concern of Kawasaki disease
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Coronary vasculitis can cause thrombosis and aneurysm and lead to MI
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Giant cell arteritis affects what arteries (4)
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Aortic arch
Vertebral Ophthalmic Temporal |
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Clinical features of Giant cell arteritis
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1- Constitutional symptoms (fever, fatigue, weight loss)
2- Tender, nodular. temporal artery 3- Ocular features- diplopia 4- Polymyalgia rheumatica |
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What is Pulseless disease?
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Takayasu Disease
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First step when you suspect Takayasu Arteritis
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Aortic Arch Angiogram
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Giant cell arteritis vs Takayasu- how to tell the difference?
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Takayasu- under 40 yrs and pulselessness of upper extremities, affect branches of aortic arch more
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What does PAN NOT affect?
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Arterioles, capillaries, venules
Pulmonary vessels No glomerulonephritis |
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PAN
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1- Arterioles, capillaries, venules not affected
2- Different stages of inflammation seen 3- Segmental, transmural, fibrinoid necrosis 4- No association with ANCA -Aneurysms, renal failure, heart, bowels, skin, testicle |
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PAN is fatal if untreated
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Notes
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Differentiate PAN and Microscopic Polyangiitis
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Microscopic Polyarteritis affects
1) Arterioles, capillaries, venules, glomerulonephritis is possible 2) Pulmonary capillarities/glomerulonephritis 3) p-ANCA present 4) inflammatory stages are of the SAME stage (either acute, subacute, chronic, repair) |
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Leukocytoclastic or hypersensitivity vasculitis (occurs in microscopic polyarteritis)
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Hypersensitivity reactions with skin lesions like purpura
Pauci immune- no or few immune deposits |
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Pauci immune means
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No or few immune deposits
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Churg-Strauss Syndrome characteristics (4)
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1- Allergic Rhinitis
2- Asthma 3- Eosinophilia 4- P-ANCA positive |
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How does Churg-Strauss kill someone?
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Coronary arteritis or myocardial infarction
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With multiple organ lesions, musculoskeletal, renal, GI, skin, peripheral NS...think about
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PAN
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4 things to remember about Wegener's
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1. necrotizing granulomatous vasculitis
2. necrotizing granulomata of tissues 3. glomerulonephritis ( focal or crescentic) 4. C-ANCA positive (90%) |
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What is Mucocutaneous Lymph Node Syndrome?
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Kawasaki Syndrome
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Clincher for diagnosis for Kawasaki Disease
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Auto Abs to endothelial and smooth muscle cells
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What is the major concern for Kawasaki Disease?
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Coronary vasculitis can cause aneurysm formation, thrombosis, and lead to an MI
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Features of Kawasaki Disease (4)
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1- Skin- desquamating rash, erythema of palms and soles
2- Mucosa- conjuctival and oral erythema 3- Lymph nodes- cervical lymphadenopathy 4- Fever |
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Buerger Disease affects what arteries?
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Tibial and Radial Arteries
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Tibial and Radial Arteries
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Buerger Disease
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Cigarette Smoking is a major risk factor in
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Buerger Disease (Thromboangiitis Obliterans)
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What is a cystic hygroma?
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Cavernous Lymphangioma- occur in the neck or axilla
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Kaposi Sarcoma is caused by
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Human Herpes Virus 8
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4 Types of Kaposi Sarcoma
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1- Chronic or classic
2- Lymphadenopathic- aggressive 3- Transplant Associated 4- AIDS associated |
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Microscopic polyangiitis/polyarteritis is also called
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Hypersensitivity or leukocytoclastic vasculitis
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Churg-Strauss Syndrome is also called
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Allergic granulomatosis and angiitis
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Kawasaki Syndrome is also called
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Mucocutaneous Lymph Node Syndrome
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Buerger Disease is also called
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Thromboangiitis obliterans
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What is hemangiosarcoma, or an angiosarcoma?
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Malignant endothelial neoplasm
Occurs in the elderly Involves skin, soft tissue, breast, liver Associated with radiation, arsenic, thorotrast, and PVC (Polyvinyl chloride) |
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When you see radiation, arsenic, thorotrast, and PVC think...
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Hemangiosarcoma
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CD 31, 34, cWF think
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Hemangiosarcoma
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Round nodules in subcutaneous tissue
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Bacillayr angiomatosis
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Main site of syphilitic aneurysm
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Thoracic aorta
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Cause of syphilitic aneurysm
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Treponema Pallidum
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Tree barking of intima of aorta
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Syphylitic aneurysm
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Most common cause of death of syphilitic aneurysm
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Cardiac failure due to valve incompetence
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Angiosarcoma
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Malignant endothelial
-Liver angiosarcoma associate with exposure to polyvinylchloride, arsenic, thorium dioxide |
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Bacillary angiomatosis
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caused by Bartonella henselae, common in AIDS
-Round nodules in subcutaneous tissues Microscope- Capillaries with eipthelioid endothelial cells |
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Kaposi's sarcoma
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Malignant tumor arising from endothelial cells or primitive mesenchymal cells
-Human Herpes Virus 8 -Raised red-purple lesions that progress from flat-->plaque-->nodule that ulcerates |
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Lymphangiosarcoma
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Malignancy of lymphatics
-Caused by long-standing chronic lymphedema (after modified radical mystectomy) |
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Pyogenic granuloma
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Vascular, red pedunculated mass that ulcerates and bleeds easily
-Trauma and pregnancy |
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Marfan's syndrome is a cause of
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Aortic dissection
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Monkenger's Arteriosclerosis
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Calcification of the media in extremities
-NO CLINICAL SIGNIFCANCE!!! |