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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
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
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
Giant Cell Arteritis
7) Diagnosis
8) Treatment
7) Temporal a. biopsy, elevation of acute phase reactants
8) Glucocorticoids: prednisone tapering over 4-6 months
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
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
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
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
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
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
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
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.
Henoch Schonlein Purpura
5) Treatment
6) Outlook
5) No indication for immunosuppression, supportive therapy of hydration, bed rest, analgesia (non-steroidal anti-inflammatory agents)