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24 Cards in this Set
- Front
- Back
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systemic sclerosis def
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Systemic sclerosis is a multisystem disease of unknown cause which falls into the scleroderma spectrum of diseases
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systemic sclerosis - clinical features
(epidemology, affected organs) |
F>M, 30-50
raynaud's phenomenon 100% skin: flexion deformity of fingers, beak like nose, digital ulcers GI: reflux, dysphagia, malabsorption from bact overgrowth, pseudoobstruction Renal: impairment, HTN Resp: PH, fibrosis Cardio: conduction, defects/arrythmias |
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systemic sclerosis - disease subtypes/pattern
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limited cutaneous scleroderma (LcSSc) 70%
= CREST diffuse cutaneous scleroderma (DcSS) 30% |
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scleroderma sine scleroderma
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systemic features without skin involvement
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scleroderma - diagnosis
(diagnostic criteria of american college of rheumatology, autoantibody pattern) |
diagnostic: by clinical features of skin and demonstration of visceral involvement (American college of rheum.)
autoantibodies: ANA 90% positive LcSSc: anticentromer Ab 70-80% DcSS: anti-scL70/anti-topoisomerase Lung: hi res CT (fibrosis) GI: barium swallow/manometry (oesophagus), barium enema (diverticulae) |
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scleroderma management general
(general principle, individual organ specific approaches) |
mainstay: organ specific symptomatic
skin: physio, lubricants oesoph: PPI, prokinetics malabsorpt: vit substitutes, rotating abx renal: ACE inhibitors!!!! PHT: vasodilators, O2, prostacycline, bosentan, pulm fibrosis: cyclophosphamide/prednisolone |
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scleroderma prognosis
(10 year prognosis, cause of death, most imporant problem to look out for) |
LcSSs: good, 70% at 10 years
main cause of death: PHT (50%) vigilance for renal hypertensive crisis: ACE I!!! |
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treatment options for raynaud's (in order of choice)
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hand warming
calcium channel blockers, ACEI bosentan sildenafil IV prostacycline digital sympathectomy ? botox |
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bosentan
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enthotheline receptor antagonist
endotheline 1 A and B receptor ind: PHT, severe raynaud's |
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scleroderma - PHT treatment options
(general, in severe cases) |
O2, vasodilators, warfarin
severe cases: IV prostacycline additional benefit: sildenafil |
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primary sjoegren's syndrome diagosis
(autoantibodies, diagnostic test) |
diagnostic: schirmer's test
(<10mm in 5 minutes) anti-Ro 70% (in primary sjoegren) ANA usually positive |
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Systemic lupus erythematodes
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SLE is an inflammatory multi system disorder with arthralgia and rashes the most common features and cerebral and renal disease the most common problems
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SLE clinical features
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general: fever, tiredness. weight loss
joints 90%: arthritis RA like but appear normal, AVN muscles: myalgia, but no myositis skin 75% "butterfly rash", discoid rashe vasculitic lesion on finger tips/nails, purpura, urticaria lungs: 50% pleural effusions, shrinking lung syndrome, pneumonitis CVS 25% pericardial effusions, myocarditis, Libman-Sachs endocarditis renal: 30% nephritis, renal vein thrombosis (APL syndrome) CNS 60% neuro/psychiatric symptoms eye: retinal vasculitis with infarcts GI: mouth ulcers, mesenteric vasculitis with bowel infarction haem: autiantibodies against PLT, erythrocytes, WBC |
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SLE variants
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chronic discoid lupus
drug induced lupus antiphospholipid syndrome |
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SLE investigations
(diagnostic, autoantibodies, routine) |
* diagnostic: simple clinical (rash)/autoantibodies present
American college criteria for research * autoantibodies ANA 100% but non-specific, anti-dsDNA specific but only in 70% antinucleosome Ab (pos before dsDNAAb), anti-Ro, anti-La, anti-RNA Complement factors low in active disease Immunoglobulins raised and polyclonal * routine tests ESR high CRP normal!!!! low WBC, PLT, Hb due to auto-antibodies disease severity: ESR, complement, anti dsDNA |
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antiphospholipid antibodies
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anti-cardiolipin antibodies 45%
anti-beta2 glycoprotein1 lupus anticoagulant |
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SLE management
(general advice, pharmacotherapy) |
avoid excessive sunlight exposure
avoid cardiac risk factors hydroxychloroquine NSAIDs corticosteroids for flare ups immunsuppressives for severe disease |
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SLE prognosis
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good prognosis. 10 year survival 90%
remission/relapsing course most frequent pattern first 10 years establish pattern arthritis is unlikly to be destructive as in RA |
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SLE and pregnancy
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not a contraindication
barrier methods better than pill recurrent miscarriages can occur due to APL syndrome usual Rx should be continued |
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antiphospholipid syndrome def
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The antiphospholipid syndrome is ssociated with autoantibodies with specifity for neg charged phospholipids.
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antiphospholipid syndrome features
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arterial/venous thrombosis
(strokes, budd-chiari, addison's) recurrent miscarriages |
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APL sydrome diagnosis
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diagnostic: anti-cardiolipin antibodies
ESR normal ANA normal |
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APL syndrome treatment
(what, when and how during pregnancy) |
anticoagulation if hx of thrombosis
high antibody levels (particularly if lupus anticoagulans or anti beta 2 GP1) warrant anticoagulation aspirin, warfarin heparin and aspirin during pregnancy |
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felty syndrome in RA
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leg ulcers
recurrent infections Rx: splenomegaly, DMARDs (gold), G-CSF |
