大四下学期11级内科血液17sle

1、系统性红斑狼疮 Systemic Lupus Erythematosus刘升云风湿免疫科郑州大学第一附属医院DefinitionA chronic inflammatory autoimmune disease of unknown etiology, with protean manifestations and a variable course and prognosis. It is characterized by periods of relative quiescence and periods of exacerbations, which may involve any or

2、gan or system in various combinations. Patients develop distinct immunologic abnormalities, in particular, antinuclear, anticytoplasmic, and antiphospholipid antibodies. 自身免疫Autoimmunity“The conquerors make war upon themselves.Blood against blood, self against self”From King Richard IIIWilliam Shake

3、speare (1592) (Perhaps the first published reference to autoimmunity)The Causes of SLEGenetic factorsEnvironmental factorsUltra-violet lightAntibiotics and Certain drugs InfectionSex hormonesPathogenesis of SLEPathologyMucous edema of the connective tissue matrix Fibrinoid degeneration of connective

4、 tissueNarcotizing vasculitisTypical lesions:Hematoxylin BodyPeriarterial Fibrosis (onionskin laminations of collagen)Myocardium, Hematoxylin Body Spleen, Periarterial FibrosisClinical FindingsConstitutional signs and symptomsMucocutaneous lesionsMusculoskeletalNephrologicalCardiovascularRespiratory

5、NeurologicalGastrointestinalHematological Antiphospholipid syndromeConstitutional signs & symptomsFeverMalaiseWeight lossClinical FindingsConstitutional signs and symptomsMucocutaneous lesionsMusculoskeletalNephrologicalCardiovascularRespiratoryNeurologicalGastrointestinalHematological Antiphospholi

6、pid syndromeMucocutaneous Lesions RashMalar erythemaTumid lupus and oral ulcer Bullous RashHand, Cutaneous LesionsLivedo reticularisHands, Digital GangreneLeg, Livedo reticularisScalp, AlopeciaClinical FindingsConstitutional signs and symptomsMucocutaneous lesionsMusculoskeletalNephrologicalCardiova

7、scularRespiratoryNeurologicalGastrointestinalHematological Antiphospholipid syndromeMusculoskeletal SystemArthritis (non-erosive)MyositisMyalgiasHand, Jaccouds Arthropathy (Clinical Appearance and Roentgenogram)Clinical FindingsConstitutional signs and symptomsMucocutaneous lesionsMusculoskeletalNep

8、hrologicalCardiovascularRespiratoryNeurologicalGastrointestinalHematological Antiphospholipid syndromeClinical Features of Patients With Lupus NephritisClinical FindingsConstitutional signs and symptomsMucocutaneous lesionsMusculoskeletalNephrologicalCardiovascularRespiratoryNeurologicalGastrointest

9、inalHematological Antiphospholipid syndromeCardiovascular SystemPericarditisMyocarditisEndocarditisCoronary artery disease Coronary vasculitisLibman-sacks EndocarditisLibman-Sacks endocarditis(H&E)Two verrucae on the surface of this valve contain fibrin and necrotic cell debris. Inflammatory cells a

10、re localized primarily at the endocardial surface (H&E).Coronary vasculitisClinical FindingsConstitutional signs and symptomsMucocutaneous lesionsMusculoskeletalNephrologicalCardiovascularRespiratoryNeurologicalGastrointestinalHematological Antiphospholipid syndromePleuropulmonary Manifestations Ple

11、uritis Pleural effusion Acute pneumonitis Chronic interstitial lung disease Diffuse alveolar hemorrhage Pulmonary arterial hypertension Shrinking lung syndrome Diffuse alveolar hemorrhageClinical FindingsConstitutional signs and symptomsMucocutaneous lesionsMusculoskeletalNephrologicalCardiovascular

12、RespiratoryNeurologicalGastrointestinalHematological Antiphospholipid syndromeNeuropsychiatric Syndromes (NPSLE)Clinical FindingsConstitutional signs and symptomsMucocutaneous lesionsMusculoskeletalNephrologicalCardiovascularRespiratoryNeurologicalGastrointestinalHematological Antiphospholipid syndr

13、omeGastrointestinal Features AnorexiaNauseaDiarrhea Protein-losing enteropathyAbdominal pain (Acute abdomen) Intestinal pseudo-obstructionClinical FindingsConstitutional signs and symptomsMucocutaneous lesionsMusculoskeletalNephrologicalCardiovascularRespiratoryNeurologicalGastrointestinalHematologi

14、cal Antiphospholipid syndromeHematological System AnemiaHemolytic Shortened lifespan of RBCNutritional Kidney diseasesLeukopeniaThrombocytopeniaAnti-platelet antibodiesAntiphospholipid antibodiesThrombotic thrombocytopenic purpuraClinical FindingsConstitutional signs and symptomsMucocutaneous lesion

15、sMusculoskeletalNephrologicalCardiovascularRespiratoryNeurologicalGastrointestinalHematological Antiphospholipid syndromeAntiphospholipid syndromeRecurrent arterial or venous thrombosisRecurrent spontaneous abortionsThrombocytopeniaPresence of antiphospholipid (aPL) antibodies in the blood“Catastrop

16、hic antiphospholipid antibody syndrome”Antiphospholipid syndromeOthers Sjgren syndromeOphthalmologic diseasesInternal Medicine Is a Branch of Lupus -Morris Ziff Dallas 1990Laboratory InvestigationsGeneral:Acute phase reactive productsESR,CRPComplements: C3, C4Rational blood test and plateletAutoanti

17、bodiesImmunopathologyLupus band testRenal biopsyAntinuclear Antibodies in SLEDLE: Ig Deposition in Skin (Photomicrograph)LE CellClassification CriteriaManagementGeneral MeasuresAvoid fatigue and stressAvoid excess UV light exposureDietary manipulationCare with contraceptionPregnancy problemsSymptomi

18、c treatmentFever:Arthritis: NSAIDRash: hydroxychloroquineLow-dose corticosteroidsHeadache:Disease-control therapyCorticosteroidsHydroxychloroquine Immunosuppressive therapyMycophenolate mofetil Cyclophosphamide Azathioprine Cyclosporin A Methotrexate Leflunomide Other therapiesIntravenous Immunoglobulin (IVIG) Plasma exchangeBiologic Therapies B CellDepleting Therapies Rituximab (RTX) B