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13 Cards in this Set
- Front
- Back
Giant Cell Arteritis
1) Vasculitis classification/ vasculature involved 2) aka? 3) Epidemiology |
1) Large vessel vaculitis: a. above diaphragm
-Carotid a. and extra cranial branches: temporal, occipital, opthalamic, posterior ciliary -Subclavia/axillary a. 2) Temporal arteritis 3) >50 yrs. Northern European risk (20/100,000) > African Americans and Hispanics > Asians |
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Giant Cell Arteritis
4) Pathology: What cells are involved, where are they, how do they become activated and how do they contribute to the problem? |
Basorum = primary entrance point of inflammation, located in adventitia
-Immature DC in adventitia--> activated and express CD86 (T-cell activation) and secrete IL18 (T-cell and monocyte recruitment) -T-cells secrete IFN gamma which stimulates macrophages and giant cells in media--> secretion of metalloproteinases, ROSs, and PDGF and VEGF, IL-6 and IL-1 -In non-stenosing arteritis, macrophages secrete FGF only into media and the internal elastic lamina remains intact -In lumen-occlusive arteritis: PDGF and VEGF --> myofibroblast migration into intima and proliferation |
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Giant Cell Arteritis
5) Clinical Manifestations 6) Laboratory abnormalities |
5) Constitutional: fatigue, wt loss, fever indicative of chronic cytokine generation.
Headach: 66%, mostly temporal but frontal/occipital pain also common Jaw pain (claudication): 50% Visual loss: acute onset partial or complete loss 15-20% opthalmic a. Arm claudication- 15% 6) Elevated Acute Phase Reactants: ESR, C-reactive protein, IL-6 |
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Giant Cell Arteritis
7) Diagnosis 8) Treatment |
7) Temporal a. biopsy, elevation of acute phase reactants
8) Glucocorticoids: prednisone tapering over 4-6 months |
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Polyarteritis Nodosa
1) Vasculitis classification/vasculature involved 2) Classic characteristics 3) Epidemiology 4) Association with Hep B? |
1) Medium-sized vessel arteritis: a. below diaphragm; superior mesenteric, celiac, hepatic, renal, muscular a. of extremities
2) necrotizing arteritis, fibrinod necrosis, increased risk for aneurysm 3) 20-70 yrs, no racial predilection, incidence = 2-4/100,000/yr but in areas endemic with Hep B 70-80/100,000 4) With Hep B, vasculitis occurs in first 6 months of infection, usually positive for surface and e antigen |
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Polyarteritis Nodosa
5) Pathology: Parts of artery involved? 6) Clinical manifestations |
5) Majority of vessel involved in necrosis/aneurysm formation. Can look red due to fibrin deposition
6) Constitutional: fatigue, wt loss, fever GI: abdominal pain from ischemia Kidney: hypertension b/c renin, angiotensis release. On biopsy spares glomerulus (b/c a. too small) PNS: mononeuritis: wrist/foot drop, ischemia of nerves (ex. radial) Skin: Nodules or ulcers Digital gangrene |
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Polyarteritis Nodosa
7) Diagnosis 8) Treatment |
7) Clinically plus biopsy and imaging (arteriogram) of involved organs/arteries. Will see both attenuations of a. (indicative of ischemia) as well as ballooning areas (indicative of necrosis)
8) 5 yr survival untreated = 13% Txt with Prednisone and oral cyclophosphamide (alkylating immunosuppressant) for one year. If HBV+ treat with IFN alpha |
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Wegener's Granulomatosis
1) Vasculitis classification/vasculature involved 2) Characteristics of granuloma 3) Epidemiology |
1) Small vessel arteritis: 3 classes of vasculature involved:
-Upper respiratory tract arterioles/capillaries -Lung arterioles and capillaries -Kidney: Glomerulonephritis (pauci immune--> no Ab/complement deposition) 2) Aggregation/nodule of macrophages and giant cells surrounded by rim of lymphocytes (Th1). Often central necrotic area 3) 25-60 yrs, prevalence 5-7/100,000, less common than giant cell |
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Wegener's Granulomatosis
4) Clinical manifestations -by system -Labs |
a) Upper respiratory tract: chronic sinusitis, chronic otitis
b) Lower respiratory tract: pulmonary nodules, alveolar hemorrhage (hemoptysis, coughing up blood). No effect on gas exchange c) Kidney: nicrotizing glomerulonephritis, lumen occluded, tuft necrosis, tubules spared d) PNS: Mononeuritis multiplex e) Skin: purpura, do not blanch, small vessels in dermis w/ extravasation of RBC Labs ANCA: Anti-Neutrophil Cytoplasmic Antibody C-ANCA: cytoplasmic reactivity Antigenic target (auto Ag) = Proteinase 3, PR3 a serine proteinase of lysosomal granules of monocytes and PMNs, indicative of Wegener's |
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Wegener's Granulomatosis
5) Mortality/morbidity 6) Treatment |
5) Treated: 80% alive at 8 yrs, untreated: only 10% after 2 yrs.
Permanent renal insufficiency: 42% End-stage renal disease: 11% Hearing loss: 35% Nasal deformities: 28% Tracheal stenosis: 13% 6) Prednisone plus cyclophophamide for 1 yr. Same as Polyarteritis Nodosa. 85-90% response rate, 75% complete remission, 30-50% 1+ relapse |
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Henoch Schonlein Purpura
1) Vasculitis classification/vasculature involved 2) Epidemiology |
1) Small vessel immune complex mediated arteritis:
GI tract: submucosal arterioles/venules Kidney: glomerulonephritis Skin: Dermal artieroles/venules 2) 5-7 yrs! (range 5-15) Incidence in children: 20/100,000 with 50% preceded with viral infection. Adults <1/100,000. Male/female: 1.5/1 |
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Henoch Schonlein Purpura
3) Clinical manifestations 4) Pathogenesis |
GI: abdominal pain, intussusception (telescoping of colon --> obstruction)
Kidney: Hematuria/proteinuria, renal insufficiency infrequent (lumen still open in glomerulus), unlike Wegener's Skin: purpura in buttocks and proximal lower extremities is characteristic Joints: arthralgia/arthritis 4) Tissue(vascular) deposition of IgA containing immune complexes into vessel wall, maybe IgA inresponse to viral infection. |
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Henoch Schonlein Purpura
5) Treatment 6) Outlook |
5) No indication for immunosuppression, supportive therapy of hydration, bed rest, analgesia (non-steroidal anti-inflammatory agents)
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