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Living with LupusLiving with Lupus
Lara GibbsLara Gibbs
QuickTime™ and a
TIFF (Uncompressed) decompressor
are needed to se...
Aims and ObjectivesAims and Objectives
• What is Lupus?What is Lupus?
• Detailed case historyDetailed case history
• Gain ...
What is SLE?What is SLE?
• Connective Tissue Disease - Autoimmune aetiologyConnective Tissue Disease - Autoimmune aetiolog...
What is SLE ctd…What is SLE ctd…
Clinical Features of SLEClinical Features of SLE
 Extremely variableExtremely variable
...
Organ SystemOrgan System Clinical FeatureClinical Feature
General FeaturesGeneral Features • Malaise and FeverMalaise and ...
Case History - JennyCase History - Jenny
• Generally not a well child - often the ill siblingGenerally not a well child - ...
Case History ctdCase History ctd
• Moved to adult hopsital, still no diagnosisMoved to adult hopsital, still no diagnosis
...
Time of LifeTime of Life SymptomsSymptoms
TeenagerTeenager
• Reaction to steroids - bloatingReaction to steroids - bloatin...
Flares
• Initiated by:Initiated by:
• RandomRandom
• Colds/VirusesColds/Viruses
• PregnancyPregnancy
• Pre-menstrualPre-me...
Daily MedicationDaily Medication
• HydroxychloroquineHydroxychloroquine
• BenzoflumethiazideBenzoflumethiazide
• Mirtazepi...
General ObservationsGeneral Observations
• SteroidsSteroids
• Patients know their diseasePatients know their disease
• ““T...
General Observations ctd…General Observations ctd…
• Being immunosuppressedBeing immunosuppressed
• Jenny has taught me no...
Any Questions?Any Questions?
ReferencesReferences
1.1. Kumar P, Clark M. Clinical Medicine Sixth Edition. 2005 ElsevierKumar P, Clark M. Clinical Medic...
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SLE: Case Presentation Slide 1 SLE: Case Presentation Slide 2 SLE: Case Presentation Slide 3 SLE: Case Presentation Slide 4 SLE: Case Presentation Slide 5 SLE: Case Presentation Slide 6 SLE: Case Presentation Slide 7 SLE: Case Presentation Slide 8 SLE: Case Presentation Slide 9 SLE: Case Presentation Slide 10 SLE: Case Presentation Slide 11 SLE: Case Presentation Slide 12 SLE: Case Presentation Slide 13 SLE: Case Presentation Slide 14
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SLE: Case Presentation

  1. 1. Living with LupusLiving with Lupus Lara GibbsLara Gibbs QuickTime™ and a TIFF (Uncompressed) decompressor are needed to see this picture.
  2. 2. Aims and ObjectivesAims and Objectives • What is Lupus?What is Lupus? • Detailed case historyDetailed case history • Gain an understanding of what it is like to live with itGain an understanding of what it is like to live with it every dayevery day
  3. 3. What is SLE?What is SLE? • Connective Tissue Disease - Autoimmune aetiologyConnective Tissue Disease - Autoimmune aetiology • Inflammatory multisystem disorderInflammatory multisystem disorder • Pathogenesis:Pathogenesis:  Failure to clear apoptotic (DNA and histones) material efficiently…Failure to clear apoptotic (DNA and histones) material efficiently…  Stimulates antibodies directed against nuclear antigens (Ro + La)Stimulates antibodies directed against nuclear antigens (Ro + La)  Formation of auto antibodies due to proteases such as granzyme BFormation of auto antibodies due to proteases such as granzyme B which are activated in apoptosis.which are activated in apoptosis.  Auto antibodies then cause tissue damage via a number ofAuto antibodies then cause tissue damage via a number of mechanismsmechanisms
  4. 4. What is SLE ctd…What is SLE ctd… Clinical Features of SLEClinical Features of SLE  Extremely variableExtremely variable  Most are consequences of vasculitisMost are consequences of vasculitis  Mild cases could present with arthralgia and fatigue onlyMild cases could present with arthralgia and fatigue only  More severe cases:More severe cases: Fever - common in flaresFever - common in flares Malaise and TirednessMalaise and Tiredness Potential multi-organ involvementPotential multi-organ involvement QuickTime™ and a TIFF (Uncompressed) decompressor are needed to see this picture. QuickTime™ and a TIFF (Uncompressed) decompressor are needed to see this picture.
  5. 5. Organ SystemOrgan System Clinical FeatureClinical Feature General FeaturesGeneral Features • Malaise and FeverMalaise and Fever • DepressionDepression MusculoskeletalMusculoskeletal • ArthralgiaArthralgia • Jaccoud’s ArthropathyJaccoud’s Arthropathy • Myalgia/MyositisMyalgia/Myositis SkinSkin • Butterfly Rash and other rashesButterfly Rash and other rashes • PhotosensitivityPhotosensitivity • AlopeciaAlopecia • Mucosal UlcerationMucosal Ulceration • Raynaud’s phenomenonRaynaud’s phenomenon CardiovascularCardiovascular • PericarditisPericarditis • MyocariditsMyocaridits • EndocariditisEndocariditis • ThromboembolismThromboembolism • Accelerated AtherosclerosisAccelerated Atherosclerosis PulmonaryPulmonary • PleurisyPleurisy • Pleural EffusionsPleural Effusions • Interstitial FibrosisInterstitial Fibrosis RenalRenal • Proteinuria/Nephrotic syndromeProteinuria/Nephrotic syndrome • HaematuriaHaematuria • GlomerulonephritisGlomerulonephritis HaematologicalHaematological • LeucopeniaLeucopenia • AnaemiaAnaemia • ThrombocytopaeniaThrombocytopaenia NeurologicalNeurological • HeadachesHeadaches • SeizuresSeizures • PsychosisPsychosis • Mononeuritis multiplexMononeuritis multiplex GastrointestinalGastrointestinal • Mesenteric VasculitisMesenteric Vasculitis • Autoimmune hepatitisAutoimmune hepatitis ImmunologicalImmunological • Immune paresisImmune paresis ReproductiveReproductive • Recurrent miscarriagesRecurrent miscarriages • Premenstrual flaresPremenstrual flares • Pregnancy flaresPregnancy flares • Neonatal Lupus with congenital heartNeonatal Lupus with congenital heart blockblock QuickTime™ and a TIFF (Uncompressed) decompressor are needed to see this picture.
  6. 6. Case History - JennyCase History - Jenny • Generally not a well child - often the ill siblingGenerally not a well child - often the ill sibling • Presented aged 14 with painful, stiff joints - mainly wristsPresented aged 14 with painful, stiff joints - mainly wrists • Skin - rash with nasty blemishes on arms and handsSkin - rash with nasty blemishes on arms and hands • Admitted to Children’s Orthopaedic hospital for testsAdmitted to Children’s Orthopaedic hospital for tests • Became constitutionally unwell:Became constitutionally unwell: More severe rashMore severe rash Fever - hot and cold sweatsFever - hot and cold sweats Feinting frequentlyFeinting frequently WeakWeak QuickTime™ and a TIFF (Uncompressed) decompressor are needed to see this picture.
  7. 7. Case History ctdCase History ctd • Moved to adult hopsital, still no diagnosisMoved to adult hopsital, still no diagnosis • Blood tests every day, staff and family very worried.Blood tests every day, staff and family very worried. • Diagnosis made:Diagnosis made: Systemic Lupus ErythematosusSystemic Lupus Erythematosus • Immediately put on a high dose of steroidsImmediately put on a high dose of steroids • Sent home after 5 months in hospitalSent home after 5 months in hospital
  8. 8. Time of LifeTime of Life SymptomsSymptoms TeenagerTeenager • Reaction to steroids - bloatingReaction to steroids - bloating • Late puberty (menarche at 17)Late puberty (menarche at 17) • Pre-menstrual flaresPre-menstrual flares • Unable to attend school or workUnable to attend school or work Young AdultYoung Adult • Pregnancy flares; Joints, Skin and Blood.Pregnancy flares; Joints, Skin and Blood. • Very worrying, closely monitoredVery worrying, closely monitored • Butterfly rashButterfly rash • Remained on steroids throughoutRemained on steroids throughout • Miscarriage x 1Miscarriage x 1 • Advised not to have second child.Advised not to have second child. • Sterilised after second childSterilised after second child AdultAdult • Continuous flares (see next slide)Continuous flares (see next slide) • Operation on hands: Jaccoud’s arthropathy (above)Operation on hands: Jaccoud’s arthropathy (above) • Stomach ulcers/bleeds (NSAIDs)Stomach ulcers/bleeds (NSAIDs) • HypothyroidismHypothyroidism • Severe photosensitivitySevere photosensitivity • Susceptible to cold/flu viruses - often initiate flaresSusceptible to cold/flu viruses - often initiate flares NowNow • Depression - 6 yearsDepression - 6 years • Pulmonary fibrosisPulmonary fibrosis • Regular follow upRegular follow up QuickTime™ and aTIFF (Uncompressed) deare needed to see this pic
  9. 9. Flares • Initiated by:Initiated by: • RandomRandom • Colds/VirusesColds/Viruses • PregnancyPregnancy • Pre-menstrualPre-menstrual • SunSun • Involve:Involve: • Fever - hot and cold sweatsFever - hot and cold sweats • ““Feel like I’m dying”Feel like I’m dying” • Skin rashes - butterfly rash (see picture - not jenny)Skin rashes - butterfly rash (see picture - not jenny) • DepressionDepression • Joint pain - “sometimes a constant ache, sometimes excruciating”Joint pain - “sometimes a constant ache, sometimes excruciating” • Blood - Leucopaenia (lymphocytic)Blood - Leucopaenia (lymphocytic)
  10. 10. Daily MedicationDaily Medication • HydroxychloroquineHydroxychloroquine • BenzoflumethiazideBenzoflumethiazide • MirtazepineMirtazepine • AmitriptylineAmitriptyline • MetoclopramideMetoclopramide • EsomeprazoleEsomeprazole • PrednisolonePrednisolone • CalcichewCalcichew • DihydrocodeineDihydrocodeine • LevothyroxineLevothyroxine • Sun blockSun block • InhalersInhalers • GavisconGaviscon QuickTime™ and a TIFF (Uncompressed) decompressor are needed to see this picture.
  11. 11. General ObservationsGeneral Observations • SteroidsSteroids • Patients know their diseasePatients know their disease • ““The depression is the worst thing to deal with”The depression is the worst thing to deal with” • ““My lungs are what makes me most angry”My lungs are what makes me most angry” • When I get ill I don’t always think it’s Lupus, I think I’m justWhen I get ill I don’t always think it’s Lupus, I think I’m just unlucky but I guess everything is connected to SLE”unlucky but I guess everything is connected to SLE” • Prognosis and characterPrognosis and character
  12. 12. General Observations ctd…General Observations ctd… • Being immunosuppressedBeing immunosuppressed • Jenny has taught me not to take my health for grantedJenny has taught me not to take my health for granted
  13. 13. Any Questions?Any Questions?
  14. 14. ReferencesReferences 1.1. Kumar P, Clark M. Clinical Medicine Sixth Edition. 2005 ElsevierKumar P, Clark M. Clinical Medicine Sixth Edition. 2005 Elsevier Saunders.Saunders. 2.2. https://www.ole.bris.ac.uk/webapps/portal/frameset.jsp?https://www.ole.bris.ac.uk/webapps/portal/frameset.jsp? tab=courses&url=/bin/common/course.pl?course_id=_45039_1 =tab=courses&url=/bin/common/course.pl?course_id=_45039_1 = eTutorial by Dr Robert MarshalleTutorial by Dr Robert Marshall 3.3. http://images.google.co.uk/images?http://images.google.co.uk/images? hl=en&q=Lupus&btnG=Search+Images&gbv=2hl=en&q=Lupus&btnG=Search+Images&gbv=2
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