Term
| Systemic Lupus Erythematous (SLE) |
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Definition
| chronic, autoimmune inflammatory dx that can affect any organ system & presents with a large spectrum of symptoms, with highly variable manifestations & follows a relapsing & remitting course; varies from relatively benign to progressive and fatal dx |
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Term
| hormonal factors believed to lead to SLE |
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Definition
| androgen, estrogen, prolactin |
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Term
| possible environmental triggers of SLE |
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Definition
| UV light, pharmaceuticals, chemicals, aromatic amines, environmental estrogens, & infections |
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Term
| genetic factors believed to cause SLE |
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Definition
| human leukocyte antigens (HLA-A1, HLA-B8, HLA-DR3) |
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Term
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Definition
| development of autoantibodies targeting "self"; environmental triggers cause cellular destruction exposing cellular nuclear components; autoimmune B lymphocytes are activated; formation of immune complexes; defective clearance of immune-complex leads to inflammatory rxns & subsequent tissue damage |
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Term
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Definition
| 1) presence or absence of ANAs; 2) requires 4 or more of 11 criteria: malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, antinuclear antibodies |
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Term
| Clinical Manifestations of SLE |
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Definition
| constitutional sx: fatigue, fever, weight changes; Musculoskeletal: arthralgia, myalgia, arthritis, major & minor joints, recurrent & short in duration; Skin: malar (butterfly) rash, discoid rash, photosensitivity; Renal: often asymptomatic but most life-threatening, develops in 1st few yrs of dx onset, regularly monitor fcn; Hematological: anemia of chronic inflammation, hemolytic anemia, leukopenia, thrombocytopenia; Cardiac: increased risk of atherosclerosis; CNS: psychosis, depression, anxiety, seizures, stroke, peripheral neuropathy, cognitive impairment |
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Term
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Definition
| 1) management of symptoms during dx flares; 2) maintenance of remission & minimizing risk of flares |
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Term
| Non-pharmacological Tx of SLE |
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Definition
| limit exposure to sunlight & use sunscreen daily; stop smoking; maintain balanced routine of rest & exercise; stress management |
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Term
| Treatment recommendations for Mild SLE |
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Definition
| control with low-potency meds or short-course steroids |
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Term
| Treatment recommendations for Severe SLE |
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Definition
| use more aggressive immunosuppressive meds |
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Term
| NSAIDs used to treat SLE (ibuprofen, ketoprofen, naproxen, indomethacin, meloxicam) |
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Definition
| choice of drug is empirical; MoA: inhibits inflammatory rxns & pain by decreasing prostaglandin synthesis; Indications: mild dx, provides symptomatic relief; Dose: anti-inflammatory dose (higher than analgesic dose); ADRs: GI bleed, hepatic toxicity, renal toxicity, HPN; Monitoring: dark/tarry stools, dyspepsia, N/V, abdominal pain, edema |
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Term
| hydroxychloroquine (Plaquenil), chloroquine (Aralen) |
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Definition
| antimalarial drugs; MoA: immunomodulator; Indications: mild dx, prevention of dx flare; Dose: 200-400 mg PO daily, 250-500 mg PO daily, gradual taper after 1-2 yrs of tx; ADRs: HA, insomnia, rashes, dermatitis, pigment changes, GI disturbances, reversible ocular toxicities; Monitor: ophthalmologic eval every 3 months with C, every 6-12 months with H; Max effect occurs in 1-3 months with C, 3-6 months in H; best used for long term management |
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Term
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Definition
| corticosteroid; MoA: decreases inflammation by suppressing migration of PMNs & Ab production; Indications: mild to severe SLE; Mild Doses: low dose <1 mg/kd/day PO or 10-20 mg PO daily; Severe dx: high dose 1-2 mg/kg PO daily; taper to minimal dose once adequate suppression is achieved to maintain dx suppression |
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Term
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Definition
| corticosteroid; MoA: decreases inflammation by suppressing migration of PMNs & Ab production; I: management of mild to severe SLE; Dosing: for life-threatening dx: pulse therapy 500-1000 mg IV qd x 3-6 days, followed by high dose prednisone PO 1-1.5 mg/kd/day tapered rapidly to low dose therapy; ADRs: infection, GI disturbances, rapid increases in BP, arrhythmias, seizures, sudden death |
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Term
| ADRs of Corticosteroids - prednisonse, methylprednisolone |
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Definition
| HPN, hyperglycemia, hyperlipidemia, hypokalemia, osteoporosis, avascular necrosis, cataracts, weight gain, infections, fluid retention |
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Term
| Monitoring Parameters for Corticosteroids |
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Definition
| BP, glucose, K (3-6 months), cholesterol (yearly), bone density (yearly) |
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Term
| cyclophosphamide (Cytoxan) |
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Definition
| cytotoxic drug; Indication: severe dx, severe lupus nephritis; MoA: alkylating agent but mechanism is unknown in SLE; Dose: 0.5-1.0 g/m^2 IV monthly for 6 months, then every 3 months for 2 yrs or for 1 yr after remission OR 1-3 mg/kg PO daily; use with steroid therapy for tx of severe lupus nephritis; intermittent IV therapy preferred over oral daily therapy b/c of reduced ADRs; ADRs: myelosuppression, hemorrhagic cystitis, 2ndary infertility, malignancy, opportunistic infections, sterility, teratogenesis; Monitor: CBC, platelet count, UA, & cytology |
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Term
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Definition
| cytotoxic drug; Indication: severe dx most commonly used in severe lupus nephritis, tx early-onset & less-severe nephritis; MoA: antimetabolite, may decrease proliferation of immune cells resulting in lower autoimmune activity; Dose: 1-3 mg/kg PO qd often in combo with steroids; ADRs: myelosuppression, hepatotoxicity, malignancy, opportunistic infections; Monitoring: CBC & platelet count & AST/ALT |
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Term
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Definition
| cytotoxic drug; Indication: severe renal & non-renal lupuse refractory to conventional cytotoxic agents; MoA: inhibits inosine monophosphate dehydrogenase (IMPDH) & suppresses de novo purine synthesis of lymphocytes inhibiting Ab production; Dose: 1-3 g PO daily; ADRs: myelosuppression, hepatotoxicity, malignancy, opportunistic infection; Monitor: CBC, LFT, & renal fcn test; more effective in preventing renal flares, higher relapse rate |
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Term
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Definition
| avoid estrogen therapies (encourage progesterone therapies); aggressive BP & lipid goals may help prevent CAD or renal dx progression; ACE-Is &/or ARBs may be useful in renal dx; calcium, vitamin D, & prophylactic bisphosphonates may reduce risk of corticosteroid-induced osteoporosis |
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Term
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Definition
| caused by hydralazine (dose related, >100 mg/day) and procainamide (presents from 1 month to several yrs of therapy); slow acetylators at higher risk; Suspected when: exposure to suspected drug, development of ANAs & at least 1 clinical feature of SLE, rapid improvement of symptoms following drug d/c; most commonly show musculoskeletal symptoms; may try NSAIDs to treat symptoms |
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