系統(tǒng)性紅斑狼瘡留學生上課

1、系統(tǒng)性紅斑狼瘡留學生上課SLEnAutoimmune disease that affects multisystemsn1.5 million cases of lupusnPrevalence of 17 to 48 per 100,000 populationnWomen Men - 9:1 ration90% cases are womennAfrican Americans WhitesnOnset usually between ages of 15 and 45 years, butnCan occur in childhood or later in lifeClinical

2、Manifestations nFor 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%, sensitivity 75%)nIt is important to remember that a patient may have SLE and no

3、t have 4 criteria.CriterianButterfly rashnDiscoid lupusnPhotosensitivitynOral ulcersnArthritisnSerositis7. Neurologic d/o8. Hematologic d/o9. Renal d/o10.Immunologic: anti-DNA, anti-Sm, false pos STS11.Anti-nuclear antibodyCutaneousnMost common rash is photosensitive, raised erythematous malar rash.

4、 55-85% develop at some point in diseasenDiscoid Lupus Erythematosus (DLE): 15-30% circular, scaly hyperpimented lesions with erythematous rim, atrophic centercan be disfiguringnMouth/vaginal/nasal ulcersnAlopecia: may be diffuse or patchy. Occurs 50%Malar RashDiscoid RashOral UlcersMSKnPolyarthriti

5、s, mild to disabling, occurs most frequently in hands, wrists, knees. Occurs 90%nJoint deformities occur in only 10%nArthritis of SLE tends to be transitory nIf single joint has persistent pain, consider osteonecrosis (prevalence increased in SLE over general population, especially if on steroids.)n

6、Myositis with elevated CK and weakness rarely occursArthritisSerositis - PulmonarynPleuritis with or without effusion- if case is mild, tx: NSAIDS- if case is severe, tx: steroidsnLife-threatening manifestations: interstitial inflammation which can lead to fibrosis and intra-alveolar hemorrhage. nAl

7、so pneumothorax and pulmonary HTN can occurSerositis CardiacnPericarditis: most common cardiac manifestation and usually responds to NSAIDs. nMyocarditis (rare) and fibrinous endocarditis (Libman-Sacks) may occur. Steroids plus treatment for CHF/arrhythmia or embolic events. nMI due to atheroscleros

8、is can occur in 35 y/oNeuronCranial or peripheral neuropathy occurs in 10-15%, it is probably secondary to vasculitis in small arteries supplying nerves. nDiffuse CNS dysfunction: memory and reasoning difficultynHeadache: if excruciating, often indicate acute flarenSeizures of any typenPsychosis: mu

9、st 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.nTIA, Stroke: mostly increased among patients that are APLA positiven50-fold increase in risk of vascular events in women under 45 compare

10、d to healthy womennTreatment for clotting event is long-term anticoagulationHemenAnemia: usually Normochromic, normocyticnLeukopenia: almost always consists of lymphopenia, not granulocytopenianThrombocytopenia RenalnNephritis: usually asymptomatic, so always check UA if patient has known or suspect

11、ed SLE nOccurs early in course of disease-if not present w/in 1 yr, probably will not occur.nHistologic classification by renal biopsy is useful to plan therapy Histologic ClassificationsnClass I is minimal mesangial glomerulonephritis which is histologically normal on light microscopy but with mesa

12、ngial deposits on electron microscopy.nClass II is based on a finding of mesangial proliferative lupus nephritis. This form typically responds completely to treatment with corticosteroids.nClass III is focal proliferative nephritis and often successfully responds to treatment with high doses of cort

13、icosteroids.nClass IV is diffuse proliferative nephritis. This form is mainly treated with corticosteroids and immunosuppressant drugs.nClass V is membranous nephritis and is characterized by extreme edema and protein loss.nClass VI GlomerulosclerosisImmunoglobulinsnAnti-dsDNA IgG: very specific, ma

14、y correlate with disease activitynAnti-Sm: specific, but only present in 25% of cases, does not correlate with activitynAPLA: not specific. Used to identify patients at increased risk for clots, thrombocytopenia and fetal lossANAnANA: positive in 95% of cases. Pretest probability affects interpretat

15、ion. In PCP setting, 2% for SLE. In rheum: 30%nLow Positive (1:160 or lower): SLE likelihood 2% (26% for rheumatologists) nHigh Positive (1:320 or higher): SLE likelihood: 2-17% (32-81% for rheumatologists)nSLE specific patterns: Rim and HomogenousAdditional work-up-Serum cr. and albumin-CBC w/ diff

16、-U/A-ESR-Complement levels-Renal bx if warranted TreatmentnTreatment plans are based on patient age, sex, health, symptoms, and lifestylenGoals of treatment are to: -prevent flares -treat flares when they occur -minimize organ damage and complicationsConservative managementnFor those w/out major org

17、an involvement. nNSAIDs: to control pain, swelling, and fevernCaution w/ NSAIDS though. SLE pts are at increased risk for aseptic meningitis nAntimalarials: Generally to treat fatigue joint pain, skin rashes, and inflammation of the lungsnCommonly used: HydroxycholorquinenUsed alone or in combinatio

18、n with other drugsCont.nCorticosteroids (Mainstay of SLE treatment)nTo rapidly suppress inflammationnUsually start with high-dose IV pulse and convert to PO steroids with goal of tapering and converting to something else.nCommonly used: prednisone, hydrocortisone, methylprednisolone, and dexamethasoneImmunosuppressivesnPrimarily for CNS/renal involvementnMycophenolate mofetil (cellcept)nAzathioprine (imuran): requires several mon