Systemic Lupus Erythematosus -SLE PT2.ppt

Musculoskeletal disorders: Systemic lupus erythematosus. Definition, etiology, diagnosis, treatment goal, treatment / management.

1. Systemic
Lupus
Erythematosus

2. contents
•INTRODUCTION
•EPIDEMIOLOGY
•PATHOGENISIS
•PRECIPITATING FACTORS
•DIAGNOSIS
•CLINICAL MANIFESTATIONS
•CLASSIFICATION
•TREATMENT
•CONCLUSION

3. INTRODUCTION
•SLEwasfirstdescribedin1828;itsnamehelpstodefinethe
disease.
•Systemicisbecausethediseasecanaffectorgansandtissue
throughoutthebody.
•LupusisLatinwordforwolf.Itreferstotherashthat
extendsacrossthebridgeofthenoseanduppercheekbones
(butterfly)andwasthoughttoresembleawolfbite.
•ErythematosusisfromtheGreekword
•forredandreferstothecoloroftherash
.

4. SLE can affect any part of the body, but
most often harms the
1.Heart
2.Joints
3.Skin
4.Lungs
5.Blood Vessels
6.Liver
7.Kidneys
8.Nervous System

5. EPIDEMIOLOGY
•TheprevalenceofSLEishigheramongAsians,Afro-
Americans,Afro-Caribbean’s,HispanicAmericans,
andAsianIndiansinGreatBritain.
•ThereisanincreasedfrequencyofSLEamong
women(10:1)thathasbeenattributedtoanestrogen
hormonaleffect.
•Prevalenceinmaleswas31.5and7.0per100000in
AsiansandWhitesrespectively;infemalesthefigures
were69.7and31.7per100000.

6. •SLEisrareinIndia.
•ThereportedprevalenceofSLEinIndiais3.2per
100,000population
•InIndiaonly50%-60%survivalat10years.
•TheepidemiologyofSLEvariessubstantiallybetween
differentsexandagegroupsandisdistributed
unequallyamonggeographicalregions;specifically,
SLEoccursmorefrequentlyinhigh-incomecountries.
•PossiblereasonsforpoorsurvivalinIndianSLEinclude
delayindiagnosis,referralbias(onlythemostserious
casesarereferredbypractitioners),suboptimalhealth
carefacilitiesandaninherentlymoreseveredisease.

7. PATHOGENISIS
SLEiscausedbytheinteractionsbetween
susceptibilitygenesandenvironmentalfactors,
resultinginabnormalimmuneresponses.
HyperreactivityofTandB-lymphocytesisindicated
byincreasedsurfaceexpressionofmoleculessuchas
HLA-DandCD40L

8. The end results of this abnormalities is sustained
production of pathogenic auto antibodies and formation
of immune complexes that bind target tissues resulting
in the
1.SequestrationanddestructionofIgcoatedcirculating
cells
2.Fixationandcleavingofcomplementproteins
3. Release of chemotoxins and vasoactive peptides, and
destructive enzymes into the tissues.

9. •Many autoantibodies in the persons with SLE are
directed against DNA or RNA complexes such as
nucleosomes, some nucleolar RNA.
•In individual with SLE, phagocytosis and removal of
apoptotic and of immune complexes are impaired.
•Thus in SLE, the antigens are available they are
presented in locations recognized by immune
complexes persists for prolonged periods of time,
allowing tissue damage to accumulate to the point of
clinical illness.

10. •FemalegenderispermissiveforSLEfemalesmakehigher
antibodyresponsesthanmales.
•EstradiolbindstoreceptorsonTandB-lymphocytes,
increasingactivationandsurvivalofthesecells,thusfavoring
prolongedimmuneresponses.

13. PRECIPITATING FACTORS
Exposuretothesun,fluorescentlights,ortanningbeds
Infections(Viral)
Surgery
Pregnancy
Therapeuticabortions
Stress

14. Antibody Prevalence
Antinuclearantibodies 98%
Anti-dsDNA 70%
Anti-Sm,anti-Ro,antiplatelet 30%
Anti-RNP 40%
Antihistone 70%
Antiphospholipids 50%
Antierythrocyte 60%
Antineuronal 60%
AntiribosomalP 20%
DIAGNOSIS:

15. ClinicalmanifestationsofSLE:

16. Malarrash Fixederethema,flatorraised,overthe
malareminences
Discoidrash Erythematouscircularraisedpaths
Photosensitivity exposuretouvlightcauserash
oralulcers includeoralandoesopharangealulcers
arthritis arthritisoftwoormorejoints
serositis pleuritisorpericarditis
renaldisorder protinuria>0.5g/d
neurologicaldisorder seizursorpsychosis
hematologicaldisorder heamolytic anemia, lucopenia,
thrombocytopenia
immunologicaldisorder anti-dsdna,anti-sm
antinuclearantibodies anabnormaltitreofANAby
immunoflurescenceoranequivalent
assayatanypointintimeintheabsence
ofdrugsknowntoinduceANAs

17. TREATMENT
•AsSLEisachronicdiseasewithnoknowncure,treatmentis
restrictedtodealingwiththesymptoms;essentiallythisinvolves.
•Preventingflaresandreducingtheirseverityanddurationwhen
theyoccur.
•Thereareseveralmeansofpreventinganddealingwithflares,
includingdrugs,alternativemedicineandlifestylechanges.
•CareofpatientswithSLEdependsondiseaseseverity.
•Periodicfollow-upandlaboratorytesting,includingurinalyses,are
imperativetodetectsignsandsymptomsofneworgansystem
involvementandtomonitorresponseorADRtotherapies.

18. TREATMENT
•TreatmentofSLEclassifiedas
Nonpharmacologictreatment
Pharmacologicaltreatment

19. Non Pharmacological Treatment
Diet and nutrition
•Dietary fish oil
•A balanced diet, including carbohydrates, proteins, and
fats
•Active inflammatory disease and fever may require an
increase in caloric intake.

20. •Steroids (prednisone) increase appetite
•A diet that is low in saturated fat.
•Vitamins
•Calcium
•Herbal supplements

21. •Exercise
•Immunizations
•AvoidingSLETriggers
•ReducingStress

22. Pharmacological management
•Category I (Mild SLE)-----CAN
Creamsandsunblocks
Nonsteroidalanti-inflammatorydrugs
Antimalarialsdrugs

23. •Category II (Moderate SLE)----CAPRIN
•Prednisolone
•Antimalarials
•Calcium supplements
•Intermittent use of NSAIDs
•Rifampicin + INH or INH + Ethambutol as
prophylactic for TB

24. •Category III (Severe SLE)---A CM or PM
•Azathioprine
•Plasmapheresis
•Methotrexate
•Cyclosporine
•Mycophenolate mofetil
CAN SUN CAPRIN is A My CM or PM
•Category IV (SLE with miscellaneous features)
•Treatment is based on the symptoms.

25. Drug Category:
•NSAIDSlikesalicylatesdosestowardstheirupperlimit
isbeneficial.
•Methotrexate:10-25mgonceaweekwithfolicacid.It
shouldbedecreasedwhenCrCl<25ml/min.
•Glucocorticoidsoral:
•Prednisone,Prednisolone0.5-1mg/kgbodyweightper
dayforsevereSLE.0.07-0.3mg/kgbodyweightfor
mildSLE.

26. MethylprednisoloneIV:forseveredisease1givevery
3days.
Cyclophosphamide:IV7-25mg/kgeverymonthfor6
months.ORAL1.5-3mg/kgperday
Mycophenolatemofetil:2-3g/day
Azathioprine:2-3mg/kgperdayPOdecreasefrequency
ofdoseifCrclis<50ml/min.
Hydroxychloroquine:150mg daily reduced gradually
until control is achieved. Max dose in adults is
2.5mg/kg body weight.

27. NEWER DRUGS
•HormoneTreatments
•Dehydroepiandrosterone (DHEA)
•Prasterone
•Danazol
•Plasmapheresis

28. Investigational Treatments
•Monoclonal Antibodies (MAbs)
•Epratuzumab
•Belimumab
•Rituximab
•Leflunomide

29. •Autologous Stem Cell Transplantation:
The procedure first removes the cells from the patient, who then
receives high-dose immunotherapy. The stem cells are then
reintroduced.
UVA-1 Phototherapy
A treatment which uses ultraviolet A-1 (UVA-1) radiation,
which are long UVA wave lengths that do not promote sunburn
and may actually block inflammatory immune factors

30. CONCLUSION
•PatientswithSLEhaveimprovedgreatlysincethe
diseasewasfirstdescribed.
•Milddiseasemayrequirenodrugsoronlysymptomatic
therapywithParacetamoloranNSAID.
•Hydroxychloroquineoftencontrolsmildtomoderate
SLEandmaykeepthediseaseinremission.
•Patientswithmoreactivediseasewillneedtreatment
withsystemiccorticosteroidsorcytotoxicdrugssuchas
azathioprineorcyclophosphamide.
•Manynewdrugsareunderresearchwhichwillhelpthe
bettercontroloverSLE