病案讨论systemic lupus 课件

病案討論 -Systemic Lupus Erythematosus 曾素卿 2006/09 The case nGeneral patient information Name:林 Age:40 Gender:female Education: high Marital status: married Occupation: office lady Admission date:94/04/22 Discharge date:94/04/25 Chart No:61591622 nChief complaint Progressive lower limb edema and gain of body weight for about 10 days nPresent illness () A 40-year-old woman was a case of SLE and diagnosed at 南門醫院 at age 19. She was under treatment at 東元醫院 and had received several cycles of pulse therapy. She had operation history of endometriosis s/p on MMH in 1994 and pregnancy with abortion (due to fever) for two times and an episode of interauterine death, followed by acute renal failure s/p hemodialysis for 3 months. nPresent illness () She sustained from repeated cystitis and PIP in 2001/10 and followed by HPV infected vaginitis in 2002/7. Type lupus nephritis was told from renal biopsy at 台大醫院. Mrs.Lin appeared to our clinic in 2004/2/6 with severe Raynauds phenomenom and digital vasculitic purpura which were also present over elbows, lower limbs and pretibial area. nPresent illness () She was admitted to received pulse therapy and cystitis with vaginitis treatment during 93/05/14-93/05/20. She was admitted twice to our hospital due to finger and toe tips vasculitis and malar rash (2004/5, 2004/7) and herbal medicine induce flare. nPresent illness () Due to her eager for baby, she refused treatments as pulse and cytotoxics. However, recurrent lower limb edema with heavy proteinuria despite prednisolone and azathiprine. Hydroxychloroquine was withheld due to patients fear of skin hyperpigmentation. nPresent illness () Her laboratory tests resulted in dsDNA=31.94295626185031003222356178 44.514410.940 C3/C4=25/1026/10385750 ESR=41759183725351575049 alb=2.72.53.52.0 Uprotein=2.772.055.444.7g/day nPresent illness () aCLIgG=7.817.3GPL/ml(N9.8MPL/ml(15 44.3 E.S.R.1hrmm/HR0-1285 CreatinineMG/DL0.5-1.10.30.4 Discussion SLE with lupus nephritis class with peripheral vasculitis O: Lab data(三) 24 hrs urine (1900 ml) Protein 452 MG/DL= 8.58 g/day Creatinine H 59.7 MG/DL=ClCr=196 ml/min Discussion SLE with lupus nephritis class with peripheral vasculitis nAssessment: 1.This patient is belong to WHO grade IV, diffuse proliferative lupus nephritis (DPLN) affecting 50% of glomeruli aggressive immunosuppression is recommended. If untreated, develop end-stage renal disease (ESRD) within 2 years. Discussion SLE with lupus nephritis class with peripheral vasculitis nAssessment: 2.This patients with dangerous proliferative forms of glomerular damage and proteinuria (500 mg per 24 h) therefore, aggressive immunosuppression is indicated (usually systemic glucocorticoids plus a cytotoxic drug) but proteinuria is less likely to improve on lupus nephritis immunosuppressive therapies. Lupus nephritis tends to be an ongoing disease, with flares requiring re-treatment over many years. Discussion SLE with lupus nephritis class with peripheral vasculitis nAssessment: 3.Treat with monthly intravenous cyclophosphamide,500 to 1000 mg/m2 body surface area for 6 months,along with high-dose corticosteroids (usually initially pulse methylprednisolone,1000 mg/d for 3 days,followed by prednisone,40 to 60 mg/d,for the first month), may be benefit for this patient. Discussion SLE with lupus nephritis class with peripheral vasculitis nAssessment: 4. Due to her eager for baby, this patient refused treatments. Therefore consider other drugs like azathioprine or mycophenolate. Discussion SLE with lupus nephritis class with peripheral vasculitis nAssessment: 5. Azathioprine (a purine antagonist) added to glucocorticoids probably reduces the number of SLE flares and the maintenance glucocorticoid requirement; however, this approach requires several months to be effective, and cyclophosphamide is effective in a higher proportion of patients. Daily oral azathioprine may have fewer adverse effects than daily oral cyclophosphamide; Discussion SLE with lupus nephritis class with peripheral vasculitis nAssessment: 6.A recent prospective study in Chinese patients with lupus nephritis comparing daily oral mycophenolate plus prednisolone for 12 months to daily oral cyclophosphamide plus prednisolone for 6 months followed by oral daily azathioprine plus prednisolone showed good improvement in 80% of patients in both groups at 1 year of follow-up and fewer adverse effects with mycophenolate. Discussion SLE with lupus nephritis class with peripheral vasculitis nAssessment: 7.Pregnancy and lupus: this patient should avoid becoming pregnant before disease stable. Discussion SLE with lupus nephritis class with peripheral vasculitis nPlan: 1.Recommended drug treatment: pulse therapy :systemic glucocorticoids(0.52mg/kg per day orally or 1000 mg of methylprednisolone sodium succinate intravenously daily) for 3 days. maintenance therapy: combine corticosteroids(510 mg/day)and immunosuppressive drugsazathioprine (12 mg/kg/d) or mycophenolate(5001500 mg/d). Discussion SLE with lupus nephritis class with peripheral vasculitis nPlan: 2.Goal: There is no cure for SLE, and complete sustained remissions are rare. Therefore, the physician should plan to control acute, severe flares then develop maintenance strategies that suppress symptoms to an acceptable level and prevent organ damage. Discussion SLE with lupus nephritis class with peripheral vasculitis nPlan: 3.Monitoring parameters: It is useful to follow tests that indicate the status of organ involvement known to be present during SLE flares. These might include hemoglobin levels, platelet counts, urinalysis, and serum levels of creatinine or albumin. There is great interest in identification of additional markers of disease activity. Candidates include levels of anti-dsDNA antibodies, several components of complement (C3 is most widely available), activated complement products, soluble interleukin (IL)2, and urinary monocyte chemotactic protein 1. Discussion SLE with lupus nephritis class with peripheral vasculitis nPlan: 4.Patient education Stress the importance of compliance with drug regimen and follow-up visits. When starting treatment with prednisone, inform patients about weight control, low-fat diet, and exercise. Advise patients about proper use and side effect profiles of other medications used in treating SLE. Counsel the patient about the importance of developing a social support system that will provide feedback about lupus self-management behaviors, problem solving, and alternate solution planning. Discussion SLE with lupus nephritis class with peripheral vasculitis nPlan: 5.Institute measures to prevent steroid-induced osteoporosis. .Vitamin D and calcium supplementation . Weight-bearing exercise .Bisphosphonates or HRT; unless otherwise contraindicated, alendronate and HRT can both be used. Discussion Hypoalbuminemia nS/O: 1.Lab data unitNormal range 4/194/224/23 AlbuminGM/DL3.5-5.02.01.92.2 Discussion Hypoalbuminemia nAssessment: 1.Hypoalbuminemia,a characteristic feature of the nephrotic syndrome, results from augmentation of both urinary albumin lossess and the catabolic rate of albumin. 2.This is a temporary measure, since the infused albumin only transiently increase serum albumin and is promptly excreted in the urine. Discussion Hypoalbuminemia nPlan: 1.Recommended drug treatment: 25% albumin 1BT iv drip, then furosemide(40mg) 1 amp for 3 days. Discussion Hypoalbuminemia nPlan: 2.Goal: treatment of the underlying disorder and an adequate protein diet combined with intervention aimed at reducing protein excretion may be beneficial. Discussion Hypoalbuminemia nPlan: 3.Monitoring parameters: periodic measurement of serum albumin levels. 4.Diet: an adequate protein diet. Discussion Hyperlipidemia(mixed type) nS/O: 1.Lab data unitNormal range 4/23 Total Cholesterol MG/DL130-230321 Triglyceride MG/DL35-165335 Discussion Hyperlipidemia(mixed type) nAssessmrent: 1.Lipid abnormalities including TC and TG have been described in this patients. 2.This patients are at an increased risk for atherosclerotic cardiovascular disease. Since the lipid abnormalities seen in nephrotic syndrome are associated with accelerated atherosclerosis. Discussion Hyperlipidemia(mixed type) nPlan: 1.Recommended drug treatment: Atorvastatin 10 mg qd am(without regard to time of day and with food if desired) 2.Acquire other lipoprotein level eg. LDL, HDL. Discussion Hyperlipidemia(mixed type) nPlan: 2.Goal: a. Reduce the risk of atherosclerosis in patient with SLE. b. NCEP( National Cholesterol Education Program)- ATP suggested TC level50% of glomeruli) 2.Which drug has become the standard drug used for controlling life- (a) threatening active lupus nephritis, particularly in patients whose renal biopsies show WHO grades III, IV, and V proliferative or membranoproliferative forms of nephritis? a.cyclophosphamide b.azathioprne c.mycophenolate 3.Which one is side effect of cyclophosphamide? (e) a. high rate of irreversible ovarian or testicular failure b. nausea c. malaise d. alopecia e.以上皆是 4.Wnen flare, anti-ds DNA and serum C3 will be? (c) a. high anti-ds DNA , high C3 b. low anti-ds DNA , low C3 c.high anti- ds DNA , low C3 d. high anti-ds