in the name of god
DESCRIPTION
IN THE NAME OF GOD. SYSTEMIC LUPUS ERYTHEMATOSIS (SLE). DEFINITION. Autoimmune Multisystem disease Autoantibodies and immune complexes. EPIDEMIOLOGY. Women of child-bearing years (90%) Most common age at onset: second and third decade All ages and ethnic groups Both sexes - PowerPoint PPT PresentationTRANSCRIPT
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IN THE NAME OF GOD
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SYSTEMIC SYSTEMIC LUPUS LUPUS
ERYTHEMATOSIERYTHEMATOSISS
(SLE)(SLE)
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DEFINITIONDEFINITION Autoimmune Autoimmune
Multisystem diseaseMultisystem disease
Autoantibodies and immune Autoantibodies and immune complexes complexes
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EPIDEMIOLOGYEPIDEMIOLOGY Women of child-bearing years (90%)Women of child-bearing years (90%) Most common age at onset: second and third Most common age at onset: second and third
decade decade
All ages and ethnic groupsAll ages and ethnic groups Both sexes Both sexes
Prevalence in US 10-400/100,000 Prevalence in US 10-400/100,000
Prevalence in Iran 30/100,000Prevalence in Iran 30/100,000
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PATHOGENESIPATHOGENESISS
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PATHOGENESISPATHOGENESIS
PredispositionSusceptibility Genes
InductionAutoimmunity
InjuryClinical Disease
Expansion
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GENETIC BASISGENETIC BASIS Twins:Twins:
Monozygotic 57%Monozygotic 57%Dizygotic 5%Dizygotic 5%
Familial aggregation:Familial aggregation:First degree relative 12%First degree relative 12%
HLA: DR2, DR3HLA: DR2, DR3 C1q, C2, C4C1q, C2, C4
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ENVIRONMENTALENVIRONMENTAL Ultraviolet B lightUltraviolet B light
Sex hormones Sex hormones EstrogenEstrogenAndrogen Androgen
Infectious agentInfectious agent
Drug Drug
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ApoptosisApoptosis
T-cellMacrophag
es
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ApoptosisApoptosis
DNA
Ro/ss-aSM
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ApoptosisApoptosis
T-cellMacrophag
es
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ApoptosisApoptosis
T-cellMacrophag
es
B cell
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PATHOGENESISPATHOGENESIS UV Flare of SLE in 70% of patientsUV Flare of SLE in 70% of patients
Infections:Infections:Induce B and T cells Recognize self Ag Induce B and T cells Recognize self Ag Auto AbAuto Ab
EBV:EBV: -- More common in SLE patients More common in SLE patients -- Activate B cell Activate B cell -- Amino acid sequences Mimic some on Amino acid sequences Mimic some on
DNA DNA
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PATHOGENESISPATHOGENESIS Female:Female:
Ab responses than maleAb responses than male
OCP & HRT: Risk of SLE (1.2-2 fold)OCP & HRT: Risk of SLE (1.2-2 fold)
Estradiol T & B cell Activation & Estradiol T & B cell Activation & SurvivalSurvival
Prolonged immune Prolonged immune responseresponse
Bind to
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Genetic
Immune comlexes
Auto antigen
Apoptotic Material
Immunogenic Ag
Phagocytosis
Apoptosis
Environmental Factors
Auto antibody
B cellT cell
CD4DC
Complement activity
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CLINICAL CLINICAL MANIFESTATIONMANIFESTATION
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CLINICAL CLINICAL MANIFESTATIONMANIFESTATION
ANY ORGAN CAN BE AFFECTEDANY ORGAN CAN BE AFFECTED
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SYSTEMIC SYSTEMIC MANIFESTATIONMANIFESTATION
Fatigue, Malaise, Fever, Anorexia, Fatigue, Malaise, Fever, Anorexia, Weight lossWeight loss
95%95%
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MUSCULOSKELETALMUSCULOSKELETAL Polyarthritis (95%)Polyarthritis (95%)
Most patientsMost patientsHands, Wrists, Knees Hands, Wrists, Knees Deformity 10%Deformity 10%Erosion RareErosion Rare
Weakness (25%)Weakness (25%) MyositisMyositisGlucocorticoidGlucocorticoid Antimalaria Antimalaria
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SYSTEMIC SYSTEMIC MANIFESTATIONMANIFESTATION
Pain persist in a single jointPain persist in a single joint
Ischemic necrosis of boneIschemic necrosis of bone
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CUTANEOUSCUTANEOUS(80%)(80%)
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CUTANEOUSCUTANEOUS Butterfly rash (50%):Butterfly rash (50%): -- Most common Most common -- Flare Flare
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CUTANEOUSCUTANEOUS Discoid rash (DLE) (20%)Discoid rash (DLE) (20%)
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RENALRENAL Nephritis (50%):Nephritis (50%):
Most serious manifestationMost serious manifestationU/A: any person with suspected SLEU/A: any person with suspected SLEClass III or IV:Class III or IV:
-- Microscopic hematuria Microscopic hematuria -- Proteinuria (> 500 Proteinuria (> 500
mg/24h) mg/24h) -- HTN HTN
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HEMATOLOGICHEMATOLOGIC Anemia (70%)Anemia (70%)
Chronic diseaseChronic diseaseHemolytic Hemolytic
Leukopenia (65%)Leukopenia (65%)LymphopeniaLymphopeniaInfection: rareInfection: rareNot require therapyNot require therapy
Thrombocytopenia (15%)Thrombocytopenia (15%)
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PULMONARYPULMONARY Pluritis (30%)Pluritis (30%) -- Most common Most common
Interstitial inflammationInterstitial inflammation
Pulmonary hemorrhage Pulmonary hemorrhage
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CARDIACCARDIAC Pericarditis (30%) Pericarditis (30%)
Myocarditis (10%)Myocarditis (10%)
Endocarditis (10%)Endocarditis (10%)Valvular insufficiencies Valvular insufficiencies Libman-Sacks Libman-Sacks
Ischemia Ischemia
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VASCULARVASCULAR Risk of vascular events 7-10 foldRisk of vascular events 7-10 fold TIA, Strok, MITIA, Strok, MI
Causes:Causes:APSAPSEmbolizationEmbolization
-- Carotid plaque Carotid plaque -- Libman-Sacks Libman-Sacks
VasculitisVasculitisAtherosclerosisAtherosclerosis
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GASTROINTESTINALGASTROINTESTINAL PeritonitisPeritonitis
VasculitisVasculitis
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OCULAROCULAR SiccaSicca
ConjunctivitisConjunctivitis
Retinal vasculitisRetinal vasculitis
Optic neuritis Optic neuritis
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NERVOUS SYSTEMNERVOUS SYSTEM CentralCentral PeripheralPeripheral
Other causes Other causes
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ANTIPHOSPHOLIPID ANTIPHOSPHOLIPID SYNDROMSYNDROM
Risk ofRisk of -- Clotting (arterial or venous) Clotting (arterial or venous) -- Fetal loss Fetal loss Tests:Tests: -- Anticardiolipin Anticardiolipin -- Lupus anticoagulant Lupus anticoagulant
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ANTIPHOSPHOLIPID ANTIPHOSPHOLIPID SYNDROMSYNDROM
High titer of IgG ACLHigh titer of IgG ACL - - Risk of clotting Risk of clotting Diagnosis:Diagnosis: -- One clinical One clinical -- One test (repeated 12w One test (repeated 12w
apart) apart)
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AUTOANTIBODIESAUTOANTIBODIES Most patients 3 y or more before Most patients 3 y or more before
symptomsymptom
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AUTOANTIBODIESAUTOANTIBODIES FANA:FANA:
Prevalence: 98%Prevalence: 98%Best screaming testBest screaming test
Anti-dsDNA: Anti-dsDNA: Prevalence: 70%Prevalence: 70%Specific (high titer) Specific (high titer) Correlate with disease activityCorrelate with disease activity
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AUTOANTIBODIESAUTOANTIBODIES Anti-Sm:Anti-Sm: -- Prevalence: 25% Prevalence: 25% -- Specific Specific -- No clinical correlation No clinical correlation Anti-Ro (SS-A):Anti-Ro (SS-A): -- Sicca, Neonatal lupus, Sicca, Neonatal lupus,
NephritisNephritis Antiphospholipid:Antiphospholipid: -- 50% 50% -- Criteria and APS syndrome Criteria and APS syndrome
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PATHOLOGYPATHOLOGY Class I: Mesangial lupus nephritisClass I: Mesangial lupus nephritis -- LM: NL LM: NL -- IF: Mesangial deposit IF: Mesangial deposit
Class II: Mesangial prolipherativeClass II: Mesangial prolipherative
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PATHOLOGYPATHOLOGY Class III: Focal proliferative Class III: Focal proliferative
Class IV: Diffuse proliferative Class IV: Diffuse proliferative
Class: V: MembranousClass: V: Membranous
Class: VI: Sclerotic Class: VI: Sclerotic
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DIAGNOSISDIAGNOSIS
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DIAGNOSISDIAGNOSIS Malar rashMalar rash
Discoid rashDiscoid rash
Oral ulcerOral ulcer
PhotosensitivityPhotosensitivity
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DIAGNOSISDIAGNOSIS Arthritis:Arthritis:
NonerosiveNonerosive≥ ≥ 2 or more peripheral joints2 or more peripheral joints
Serositis:Serositis:Pleuritis or pericarditis Pleuritis or pericarditis
Renal:Renal:Proteinuria > 500 mg or ≥ 3+, or cellular Proteinuria > 500 mg or ≥ 3+, or cellular castscasts
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DIAGNOSISDIAGNOSIS Neurologic:Neurologic:
Seizures or psychosis without other Seizures or psychosis without other causescauses
Hematologic:Hematologic:Hemolytic anemia orHemolytic anemia orLeukopenia (< 4000) orLeukopenia (< 4000) orLymphopenia (< 1500) orLymphopenia (< 1500) orThrombocytopenia (< 100,000)Thrombocytopenia (< 100,000)
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DIAGNOSISDIAGNOSIS Immunologic disorder:Immunologic disorder:
Anti-dsDNA, anti-Sm, antiphospholipidAnti-dsDNA, anti-Sm, antiphospholipid
Antinuclear antibodies:Antinuclear antibodies:By immunofluorescence By immunofluorescence
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DIAGNOSISDIAGNOSIS Criteria for classificationCriteria for classification
≥ ≥ 4 criteria4 criteria
Specificity: 95%Specificity: 95% Sensitivity: 75%Sensitivity: 75%
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DRUG-INDUCED LUPUSDRUG-INDUCED LUPUS MilderMilder
Rarely renal or CNS involvementRarely renal or CNS involvement
Drugs: hydralazine, procainamidDrugs: hydralazine, procainamid……
Positive ANA and Anti histone but rarely Anti-Positive ANA and Anti histone but rarely Anti-dsDNAdsDNA
Reversible Reversible
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TREATMENTTREATMENT
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TREATMENTTREATMENT No cureNo cure
Patients educationPatients education
Prophylactic measures:Prophylactic measures:Sunscreen Sunscreen Low dose aspirin for antiphospholipid Low dose aspirin for antiphospholipid Ab positiveAb positiveRoutine immunization Routine immunization
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TREATMENTTREATMENT Glucocorticoids:Glucocorticoids: -- For almost any manifestation For almost any manifestation
Immunomodulating agents:Immunomodulating agents: -- Antimalaria Fever, Arthritis, Antimalaria Fever, Arthritis,
CutaneousCutaneous Prevents flarePrevents flare -- Azathioprine Azathioprine -- Mycophenolate mofetile Mycophenolate mofetile -- Cyclophosphamide Cyclophosphamide
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CORSECORSE Range from mild to sever diseases Range from mild to sever diseases Survival:Survival: -- 95% at 5y and 78% at 20y 95% at 5y and 78% at 20y Causes of death:Causes of death: -- First decade: disease activity, Renal, First decade: disease activity, Renal,
InfectionInfection -- After: Thromboembolic After: Thromboembolic Critical:Critical: - - Nephritis, Cerebritis, Pulmonary Nephritis, Cerebritis, Pulmonary
hemorrhage, hemorrhage, Hematologic, CarditisHematologic, Carditis
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