系统性红斑狼疮留学生上课

Systemic Lupus Erythematosus,Doctor NaLiBin zhou medical college Yantai affiliated hospital,SLE,Autoimmune disease that affects multisystems1.5 million cases of lupusPrevalence of 17 to 48 per 100,000 populationWomen Men - 9:1 ratio90% cases are womenAfrican Americans WhitesOnset usually between ages of 15 and 45 years, butCan 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%, sensitivity 75%)It is important to remember that a patient may have SLE and not have 4 criteria.,Criteria,Butterfly rashDiscoid lupusPhotosensitivityOral ulcersArthritisSerositis,7. Neurologic d/o8. Hematologic d/o9. Renal d/o10.Immunologic: anti-DNA, anti-Sm, false pos STS11.Anti-nuclear antibody,Cutaneous,Most common rash is photosensitive, raised erythematous malar rash. 55-85% develop at some point in diseaseDiscoid Lupus Erythematosus (DLE): 15-30% circular, scaly hyperpimented lesions with erythematous rim, atrophic centercan be disfiguringMouth/vaginal/nasal ulcersAlopecia: may be diffuse 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 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: steroidsLife-threatening manifestations: interstitial inflammation 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 CHF/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 difficultyHeadache: if excruciating, often indicate acute flareSeizures of any typePsychosis: 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 positive50-fold increase in risk of vascular events in women under 45 compared to healthy womenTreatment for clotting event is long-term anticoagulation,Heme,Anemia: usually Normochromic, normocyticLeukopenia: almost always consists of lymphopenia, not granulocytopeniaThrombocytopenia,Renal,Nephritis: usually asymptomatic, so always 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 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 responds 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,Immunoglobulins,Anti-dsDNA IgG: very specific, may correlate with disease activityAnti-Sm: specific, but only present in 25% of cases, does not correlate with activityAPLA: not specific. Used to identify patients at increased risk for clots, thrombocytopenia and fetal loss,ANA,ANA: positive in 95% of cases. Pretest probability 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 albuminCBC w/ diffU/AESRComplement levelsRenal bx if warranted,Treatment,Treatment plans are based on patient age, sex, health, symptoms, and lifestyleGoals of treatment are to: -prevent flares -treat flares when they occur -minimize organ damage and complications,Conservative management,For those w/out major organ involvement. NSAIDs: to control pain, swelling, and feverCaution 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 lungsCommonly used: HydroxycholorquineUsed alone or in combination with other drugs,Cont.,Corticosteroids (Mainstay of SLE treatment)To rapidly suppress inflammationUsually 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,Immunosuppressives,Primarily for CNS/renal involvementMycophenolate mofetil (cellcept)Azathioprine (imuran): requires several months to be effective, eff