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24 Cards in this Set
- Front
- Back
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What are the antibodies present in SLE?
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ANA: anti-ssDNA, anti-dsDNA
Ab vs cytoplasmic components: anti-Sm, anti-Ro, anti-La antiphospholipid Abs: lupus anticoagulant, anticardiolipin |
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What are the systemic findings (and % involvement) in SLE?
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85% acute cutaneous dz
80-85% arthritis 50% renal dz 20% Raynaud's 35% neurologic cardiac pulmonary hepatic hematologic 50% ocular |
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What are the ophthalmic manifestations of SLE?
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1. cutaneous (discoid of eyelids)
2. Secondary Sjogren synd (20%) 3. scleral inflammation 4. neuro-ophthalmic lesions 5. retinal vasculopathy 6. uveitis (rare) |
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What kind of retinopathy is seen in a pt with + antiphospholipid Abs in the absence of SLE?
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Similar to retinal vasculopathy of SLE
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How does retinopathy correlate with SLE systemic activity?
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it is a marker of systemic dz activity
3% in outpts 29% in more severe dz more severe retinopathy is a/c CNS involvement and + antiphospholipid Abs 34% have lupus anticoagulant Ab 44% have anticardiolipin Ab |
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What are the stages/findings of lupus retinopathy?
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1. CWS +/- intraret. hemes (independent of HTN)
2. Severe arterial and venous thrombosis --> ischemia --> neovasc. --> vit heme 3. Lupus choroidopathy serous elevations of retina/RPE/both choroidal infarction choroidal neovasc in severe dz secondary to HTN or vasculitis |
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What is polyarteritis nodosa?
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systemic vasculitis characterized by subacute or chronic focal and episodic necrotizing inflammation of medium-sized and small muscular arteries
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PAN:
Pt demographics. |
40-60y/o
men:women = 3:1 0.7/100,000/yr no racial/geographic factors 10% are HBVSAg+ |
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What are the systemic findings in PAN?
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75% constitutional symptoms
mc symptom = mononeuritis multiplex renal involvement d/t vasculitis 1/3 = secondary HTN GI dz with small bowel ischemia cutaneous involvement Raynaud's phenomenon coronary arteritis pericarditis hematologic abnl CNS dz is rare |
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What is the incidence of ocular involvement in PAN?
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10-20%
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What are the ophthalmic findings in PAN?
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HTN retinopathy in pts with renal dz
retinal arteriolar occlusive dz chroidal infarcts with exudative RD d/t vasculitis of post. ciliary arts and choroidal vessels Elschnig spots neuro-ophthalmic: CN palsies, amaurosis, homonymous hemianopia, Horner's synd, optic atrophy scleral inflamm. PUK with scleritis can be presenting manifestation |
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What are the treatment and prognosis of PAN?
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untreated PAN 5 yr mortailty = 90%
systemic steroids reduce it to 50% cyclophosphomide gives 5 yr survival of 80% and induces long-term remission |
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What is the triad of Wegener granulomatosis?
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1. necrotizing granulomatous vasculitis of the upper and lower respiratory tract
2. focal segmental glomerulonephritis (85%) 3. necrotizing vasculitis of small arteries and veins **paranasal sinus involvement is characteristic |
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What are the presenting findings of Wegener granulomatosis?
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constitutional symptoms
sinusitis a/c bloody nasal d/c pulmonary symptoms arthritis derm involvement in 50%: purpura of lower extremities nervous system in 33%: mc mononeuritis multiplex |
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What is the incidence of ocular involvement in Wegener granulomatosis?
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15% at presentation
29-52% during the course of dz |
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What are the ocular findings of Wegener granulomatosis?
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orbital involvement - extension from sinuses
orbital pseudotumor (distinct from sinus involvement) orbital cellulitis dacryocystitis 16-38% scleritis (diffuse or necrotizing) +/- PUK 10% with other ocular involvement also have nonspecific uveitis (any kind) 5-12% retinal involvement (CWS - BRVO) 10-20% retinitis neovasc --> NVG, vit heme ischemic optic neuropathy 8-37% have vision loss |
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What are ANCAs?
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Abs vs cytoplasmic azurophilic granules of neuts and monos.
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What is c-ANCA associated with?
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sensitive and specific for Wegener granulomatosis
85-95% of ANCA in WG is c-ANCA has Ag specificity for proteinase 3 |
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What is p-ANCA associated with?
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PAN
microscopic PAN relapsing polychondritis retinal vasculitis Ag specificity against MPO |
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What is the diagnostic sensitivity of c- and p-ANCA for PAN and microPAN?
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PAN:
c-ANCA 5% p-ANCA 15% microPAN: c-ANCA 50-80% p-ANCA 40% |
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What are the treatment and prognosis of Wegener granulomatosis?
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untreated 1 yr mortality = 80%
93% of pts treated with cyclophosphamide and steroid --> remission with resolution of ocular manifestations |
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Birdshot retinochoroidopathy:
Another name? Pt demographic? HLA association? |
vitiliginous chorioretinitis
Caucasian females, N. European, >40y/o HLA-A29 (80-98%) |
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What are the 2 hypothesis of pathogenesis of birdshot?
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1. retinal autoimmunity: lymphocyte proliferation t retinal S-Ag
2. infex agent enhances expression via HLA-A29 of self-peptides to T-cells |
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What are the symptoms of birdshot?
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blurred vision
floaters nyctalopia color vision changes |
