Living with LupusLiving with LupusLara GibbsLara Gibbs

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Aims and ObjectivesAims and Objectives

• What is Lupus?What is Lupus?

• Detailed case history Detailed case history

• Gain an understanding of what it is like to live with it Gain an understanding of what it is like to live with it every dayevery day

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 B Formation 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 of Auto antibodies then cause tissue damage via a number of

mechanismsmechanisms

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 involvement Potential multi-organ involvement

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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 heart Neonatal Lupus with congenital heart blockblock

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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

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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

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

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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)

Daily MedicationDaily Medication

• HydroxychloroquineHydroxychloroquine• BenzoflumethiazideBenzoflumethiazide• MirtazepineMirtazepine• AmitriptylineAmitriptyline• MetoclopramideMetoclopramide• EsomeprazoleEsomeprazole• PrednisolonePrednisolone• CalcichewCalcichew• DihydrocodeineDihydrocodeine• LevothyroxineLevothyroxine• Sun blockSun block• InhalersInhalers• GavisconGaviscon

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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 just When 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

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

Any Questions?Any Questions?

ReferencesReferences

1.1. Kumar P, Clark M. Clinical Medicine Sixth Edition. 2005 Elsevier Kumar 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