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35 Cards in this Set
- Front
- Back
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What is an autoimmune disease?
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-body tissues are being attacked by its own antibodies
-more frequent in women (related to estrogen?) -presence of 1 autoimmune disease increases risk of others |
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What is systemic lupus erythmatosus (SLE)?
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-Collagen Vascular Disease- the clinical manifestations are multi-organ involved caused by immune dysfunction
-autoantibodies progress to the cellular nuclear component which triggers a chronic inflammatory response |
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How is lupus diagnosed?
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-If 4 of 11 criteria are documented at any time in the patient's medical history, dx can be made with 95% specificity and 85% sensitivity
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What are the most common signs/symptoms of SLE?
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Musculoskeletal
-arthralgia/mild arthritis with morning stiffness -SLE-antibodies to dsDNA |
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True or False: SLE is not static and most patients have fluctuations or flare-ups during the course of the disease.
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TRUE
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What are some cutaneous manifestations of SLE?
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-"butterfly" rash on bridge of the nose
-chronic and well-defined plaques that can lead to scarring and atrophy -vasculitis, urticaria, drug eruptions associated w/ tmt -photosensitivity -alopecia, Raynaud's, vasculitis |
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What are some renal manifestations of SLE?
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-lupus nephritis-significant cause of morbidity and mortality
(predictors of poor outcome include AA race, increased SrCr, poor initial response to immunosuppressive therapy, HTN and persistent nephrotic syndrome) -<10 years until failure if: SCr>1.5, HTN, WHO Class IV -proteinuria, hematuria, casts, nephrotic syndrome |
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What are some pulmonary manifestations of SLE?
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-pleurisy, coughing, dyspnea
-acute pneumonitis can lead to chronic interstitial fibrosis -diffuse interstitial disease -pulmonary HTN -diaphragmatic dysfunction -atelectasis -pulm hemmorhage (prophylaxis including abx for dental, genitourinary procedures and immunization) |
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What are some cardiovascular manifestations of SLE?
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-MI: 9 x more prevalent in SLE pts
-30% die from CV disease -pericarditis, endocarditis, myocarditis -long duration of SLE, steroid therapy, HTN, hypercholesterolemia, hx of antihypertensive use -valvular and other CV complications usually associated with APL antibody syndrome (inc PTT, veno-occlusive disease, abnormal titers of APL) |
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What are some neuropsychiatric and GI manifestations of SLE?
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-psychosis, depression, anxiety, seizures, stroke, peripheral neuropathy and cognitive impairment
-cognitive impairment -nonspecific GI symptoms like dyspepsia, abdominal pain, nausea and difficulty swallowing -messenteric vasculitis and venous occlusion -hepatitis and pancreatitis may occur as a result of drug therapy and adverse effects |
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What are some hematological manifestations of SLE?
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Anemia of a chronic disease is common
-hemolytic anemia -neutropenia -lymphopenia -thrombocytopenia -leukopenia |
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How is SLE diagnosed?
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1. Suspect in patients with features affecting 2-3 organ systems
2. UNLIKELY if pts with positive ANA and no organ system involvement or typical lab findings 3. Flourescent Antinuclear Antibody Test can be done |
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What does the flourescent antinuclear antibody test show?
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-peripheral dsDNA
-specled ribonucleoprotein (Sm antigen) -homogenous histone |
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Survival rates for SLE are currently:
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>96% at 5 years and >70% at 10 years
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Death from long term infliction of disease is from:
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organ dysfunction
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What are the therapy goals of SLE?
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1. remission
2. prevent end organ damage 3. maintain remission while minimizing long-term adverse effects to drug therapy |
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What are therapies of choice for SLE?
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medications which suppress immune system and inflammation
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What is the treatment for antiphospholipid syndrome?
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low dose ASA
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What is the treatment for fever, arthritis, serositis (mild disease)?
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NSAIDs
(monitor renal function and CNS for aseptic meningitis-subsides upon withdrawal) |
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What are the antimalarial drugs used to treat SLE?
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Hydroxychloroquine 200-400mg qd
Chloroquine 250-500mg qd |
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What are some side effects of antimalarials?
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-cutaneous manifestations, arthralgia, pleuritis, mild pericardial inflammation, fatigue
-cognitive dysfunction, mild anemia, leukopenia |
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Which antimalarial is safer and how long should treatment continue?
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Hydroxychloroquine is safer but takes 3-6 months for max effect
Gradual tapering after 1-2 years of treatment is recommended |
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When are corticosteroids used in treating SLE?
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Used for lupus nephritis, severe cases of CNS disease, pneumonitis, polyserositis, vasculitis, thrombocytopenia, and other severe disease side effects uncontrolled by previous drug therapy
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What dosing strategy is used with corticosteroids?
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The goal is to suppress the active disease with the lowest effective dose
10-20mg/day up to 1-2mg/kg/day then taper Pulse therapy 500mg-1gm qd f 3-5 days = quicker response and fewer steroid side effects |
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When is cyclophosphamide used in treating SLE and how is it dosed?
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Primarily used for nephritis and most efficacious when used in combination with corticosteroids
0.5-1gm/m2 q month up to 6 months then q 3 months x 2 years (or 1 year after remission) |
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What should be given to patients taking cyclophosphamide to reduce incidence of hemorrhagic cystitis?
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MESNA
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What should be monitored for when pt is taking cyclophosphamide?
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hematopoiesis suppression, opportunistic infections and N/V
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What are some characteristics of azathioprine?
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-oral agent up to 4mg/kg/day
-slightly more effective than prednisone alone -considered less toxic than cyclophosphamide but also less effective |
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True or False: Mycophenolate mofetil has been shown to be equally as efficacious as oral cyclophosphamide after 12 months, but the extended 36 month follow-up showed higher relapse rates in the mycophenolate group
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TRUE
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True or False: Mycophenolate maintenance therapy has proven to be more effective in preventing renal flares than quarterly IV pulse cyclophosphamide or daily azathioprine with few adverse events
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TRUE
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What is antiphospholipid therapy?
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Acute thrombotic event that requires standard anticoagulant therapy with heparin.
If using warfarin - often requires an INR of 3 or more if a second event occurs when anticoagulated |
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What drugs can attribute to drug induced lupus?
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procainamide, hydralazine, quinidine, methyldopa, penicillamine, INH, chlorpromazine
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How is drug induced lupus diagnosed?
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No history of idiopathic lupus, positive ANA, at least 1 clinical feature of SLE, resolution upon discontinuation
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True or False: Slow acetylators have a decreased risk of drug induced lupus.
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FALSE: slow acetylators = GREATER risk of drug induced lupus
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What are some nonpharmacologic modalities for treating SLE?
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-rest and exercise for fatigue
-avoid smoking (hydralazine) -avoid alfalfa sprouts and encourage fish oil -limit sun exposure and use sunscreen -psychosocial support -lupus foundation |
