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28 Cards in this Set

  • Front
  • Back
What are some of the criteria for diagnosing systemic lupus?
Photosensitivity,
Malar rash,
Mucosal ulcers,
Serositis (pleurisy and pericarditis),
Seizures/psychosis,
Renal disease (often dictates treatment),
Cytopenias,
Positive antinuclear antibody test,
Specific autoantibodies (anti-DNA)
Who is most at risk for getting SLE?
Non-caucasians
Women (90%): onset usually in child-bearing years with flares post-partum
Is SLE more severe in children or adults?
children
What causes the disease manifestations of SLE?
Pathogenic auto-antibodies (antibodies to cell surface antigens, anti-dsDNA, anti-phospholipid)
What can result from anti-phospholipid antibodies?
Thrombosis of arteries and veins
Spontaneous fetal loss
What are some of the laboratory manifestations of SLE?
Andi-dsDNA antibodies (very specific)
Antinuclear antibody (ANA) test (but many false positives)
Low complement levels
What was an initial use for anti-phospholipid antibodies?
Explained some false positives for syphilis
How can vascular injury occur in SLE?
By immune complex deposition (complement fixation, then adherence/infiltration of inflammatory cells, then tissue injury by release of liposomal contents, then the injured vessel wall becomes a potential site for thrombosis)

This can lead to accelerated atherosclerosis at these sites
How does SLE present?
Disease activity waxes and wanes with flares
Individual disease manifestations may appear separately and make diagnosis difficult
Fatigue accompanies active disease
What are some common musculoskeletal sequelae of SLE and corticosteroid treatment?
Muscle atrophy and weakness
Osteoporosis
Osteonecrosis
What is the major cause of death and disability in SLE patients?
Lupus nephritis; level of renal disease NEEDS to be assessed
What causes lupus nephritis and how does it progress?
Immune complex mediated
Early: hematuria, proteinuria, HTN
Advanced: nephrotic syndrome, renal insufficiency
What are the two types of lupus nephritis?
Proliferative and membranous
What is proliferative lupus nephritis?
Immune complex deposition between glomerular endothelial cells and the basement membrane, characterized by cellular infiltration
Often rapidly progressive and destructive
Nephritic syndrome
What is membranous lupus nephritis?
Immune complex deposition between the basement membrane and epithelial podocytes without the cellular infiltration
Nephrotic syndrome
Slow progression: early disease causes hyperfiltration, disguising the renal injury
What causes neuropsychiatric lupus?
Either from antibody-mediated neuronal injury or from vasculitis/thrombosis of vessels
What is entailed in the long-term management of SLE? (7)
Exercise
Weight control
Diet low in saturated fat and cholesterol
Optimal BP
Prevention of osteoporosis
Stop smoking
Control manifestations with least toxic drugs as early as possible
What does prednisone do in SLE?
Controls the constitutional symptoms
What are the short term side effects of prednisone?
infection, glucose intolerance, weight gain
What are the long-term side effects of prednisone?
HTN, accelerated atherosclerosis, aging/atrophy of skin, muscles, and bones; cataracts, diabetes
What are 7 components of the idea drug for SLE?
Anti-inflammatory
Immunosuppressive
Anti-thrombotic
Cholesterol lowering
Slows atheroma formation
Steroid sparing
Protects against steroid induced hyperglycemia
What does hydroxychloroquine do in SLE treatment?
Reduces severe flares (nephritis), slows thrombus formation, and improves serum cholesterol
What are three immunosuppressive drugs in SLE?
mycophenolate mofetil, cyclophosphamide and azathioprine
When are immunosuppressive drugs used in SLE?
For very severe disease manifestations (severe nephritis) or as steroid-sparing agents who would otherwise have to be an high dose of prednisone
What are the risk factors/side effects of the immunosuppressive drugs?
Infection and cancers
Birth defects (cyclophosphamide)
What is the general plan for drug treatment of severe SLE?
Start with a powerful but highly toxic drug (cyclophosphamide), and then switch to a better tolerated one after the disease is controlled (azathioprine, mycophenolate mofetil)
What are the three biologic agents that may eventually be used for SLE treatment ?
Rituximab
LJP 394
Belimumab
How does rituximab work?
chimeric monoclonal antibody against CD20 antigen on B-cells