1、Systemic Lupus Erythematosus Doctor NaLi Bin zhou medical college Yantai affiliated hospital SLE?Autoimmune disease that affects multisystems?1.5 million cases of lupus?Prevalence of 17 to 48 per 100,000 population?Women Men-9:1 ratio?90%cases are women?African Americans Whites?Onset usually between
2、 ages of 15 and 45 years,but?Can occur in childhood or later in life Clinical Manifestations?For the purpose of identifying patients in clinical studies,a person has SLE if 4 or more of the 11 criteria are present,serially or simultaneously,during any interval of observation.(specificity 95%,sensiti
3、vity 75%)?It is important to remember that a patient may have SLE and not have 4 criteria.Criteria 1.Butterfly rash 2.Discoid lupus 3.Photosensitivity 4.Oral ulcers 5.Arthritis 6.Serositis 7.Neurologic d/o 8.Hematologic d/o 9.Renal d/o 10.Immunologic:anti-DNA,anti-Sm,false pos STS 11.Anti-nuclear an
4、tibody Cutaneous?Most common rash is photosensitive,raised erythematous malar rash.55-85%develop at some point in disease?Discoid Lupus Erythematosus(DLE):15-30%circular,scaly hyperpimented lesions with erythematous rim,atrophic center can be disfiguring?Mouth/vaginal/nasal ulcers?Alopecia:may be di
5、ffuse or patchy.Occurs 50%Malar Rash Discoid Rash Oral Ulcers MSK?Polyarthritis,mild to disabling,occurs most frequently in hands,wrists,knees.Occurs 90%?Joint deformities occur in only 10%?Arthritis of SLE tends to be transitory?If single joint has persistent pain,consider osteonecrosis(prevalence
6、increased in SLE over general population,especially if on steroids.)?Myositis with elevated CK and weakness rarely occurs Arthritis Serositis-Pulmonary?Pleuritis with or without effusion -if case is mild,tx:NSAIDS -if case is severe,tx:steroids?Life-threatening manifestations:interstitial inflammati
7、on which can lead to fibrosis and intra-alveolar hemorrhage.?Also pneumothorax and pulmonary HTN can occur Serositis Cardiac?Pericarditis:most common cardiac manifestation and usually responds to NSAIDs.?Myocarditis(rare)and fibrinous endocarditis(Libman-Sacks)may occur.Steroids plus treatment for C
8、HF/arrhythmia or embolic events.?MI due to atherosclerosis can occur in 35 y/o Neuro?Cranial or peripheral neuropathy occurs in 10-15%,it is probably secondary to vasculitis in small arteries supplying nerves.?Diffuse CNS dysfunction:memory and reasoning difficulty?Headache:if excruciating,often ind
9、icate acute flare?Seizures of any type?Psychosis:must distinguish from steroid-induced psychosis(occurs in 1st weeks of tx at doses 40mg prednisone and resolves after several days of reducing or stopping tx)Cont.?TIA,Stroke:mostly increased among patients that are APLA positive?50-fold increase in r
10、isk of vascular events in women under 45 compared to healthy women?Treatment for clotting event is long-term anticoagulation Heme?Anemia:usually Normochromic,normocytic?Leukopenia:almost always consists of lymphopenia,not granulocytopenia?Thrombocytopenia Renal?Nephritis:usually asymptomatic,so alwa
11、ys check UA if patient has known or suspected SLE?Occurs early in course of disease-if not present w/in 1 yr,probably will not occur.?Histologic classification by renal biopsy is useful to plan therapy Histologic Classifications?Class I is minimal mesangial glomerulonephritis which is histologically
12、 normal on light microscopy but with mesangial deposits on electron microscopy.?Class II is based on a finding of mesangial proliferative lupus nephritis.This form typically responds completely to treatment with corticosteroids.?Class III is focal proliferative nephritis and often successfully respo
13、nds to treatment with high doses of corticosteroids.?Class IV is diffuse proliferative nephritis.This form is mainly treated with corticosteroids and immunosuppressant drugs.?Class V is membranous nephritis and is characterized by extreme edema and protein loss.?Class VI Glomerulosclerosis Immunoglo
14、bulins?Anti-dsDNA IgG:very specific,may correlate with disease activity?Anti-Sm:specific,but only present in 25%of cases,does not correlate with activity?APLA:not specific.Used to identify patients at increased risk for clots,thrombocytopenia and fetal loss ANA?ANA:positive in 95%of cases.Pretest pr
15、obability affects interpretation.In PCP setting,2%for SLE.In rheum:30%?Low Positive(1:160 or lower):SLE likelihood 2%(26%for rheumatologists)?High Positive(1:320 or higher):SLE likelihood:2-17%(32-81%for rheumatologists)?SLE specific patterns:Rim and Homogenous Additional work-up-Serum cr.and albumi
16、n-CBC w/diff-U/A-ESR-Complement levels-Renal bx if warranted Treatment?Treatment plans are based on patient age,sex,health,symptoms,and lifestyle?Goals of treatment are to:-prevent flares -treat flares when they occur -minimize organ damage and complications Conservative management?For those w/out m
17、ajor organ involvement.?NSAIDs:to control pain,swelling,and fever?Caution w/NSAIDS though.SLE pts are at increased risk for aseptic meningitis?Antimalarials:Generally to treat fatigue joint pain,skin rashes,and inflammation of the lungs?Commonly used:Hydroxycholorquine?Used alone or in combination w
18、ith other drugs Cont.?Corticosteroids(Mainstay of SLE treatment)?To rapidly suppress inflammation?Usually start with high-dose IV pulse and convert to PO steroids with goal of tapering and converting to something else.?Commonly used:prednisone,hydrocortisone,methylprednisolone,and dexamethasone Immu
19、nosuppressives?Primarily for CNS/renal involvement?Mycophenolate mofetil(cellcept)?Azathioprine(imuran):requires several months to be effective,effective in smaller percentage of patients?MTX:for treatment of dermatitis and arthritis,not life-threatening disease?Cyclosporine:used in steroid-resistant SLE,risk of nephrotoxicity?Cyclophosphamide(cytoxan)Almost all trials performed on patients with nephritis Thank you for your attetion!
