Patient35 years old femalePresented 2 mths ago with right shoulder painInitially treated as Reactive Arthritis –

UnrelievedAdmitted outside for sudden eruption of

rashes and oozing crusts over lips n face and epistaxis with generalized anasarca

2 seizures after dischargeNo history of medicines, allergies, family riskInfertility (Laparoscopy,IVF –Unsuccessful)k/c/o Hypothyroidism

On ExaminationObeseApyrexial, RR 20, HR 110, BP 144/70Erythematous Rash on body,Crusts n

Oozing lesions over lower lips and left ankle, Oral Ulcers

Severe Pallor, Moderate pitting pedal oedema

CVS: tachycardicResp, Abdo, neuro : normal

Laboratory investigations

Hb 6.6 gm% , Plt 96,000/cmmTLC 2200/cmmESR 47Anti-CCP NegativeINR, PTT, BT/CT : normalS.Creat = 2.1 mg%Urine R/M s/o Proteinuria + Haematuria

Laboratory investigationsHb 6.6 gm% , Plt 96,000/cmmTLC 2200/cmmESR 47Anti-CCP NegativeINR, PTT, BT/CT : normalS.Creat = 2.1 mg%Urine R/M s/o Proteinuria + HaematuriaANA,Anti-DsDNA = Positive

Treatment GivenIV Methyl PrednisoloneFollowed by Oral Prednisolone ( 1

mg/kg/day)

Current Condition of PatientAsymptomaticAmbulatoryShoulder Pain – Better Pancytopenia – Reverted back to Normal

countsRenal Function – Urine – no Proteinuria, S.Creat = 1.2 mg%

Introduction to SLEAuto-immune disorderMultisystem microvascular inflammationFormation of autoantibodiesChronic with relapsing and remitting course

EtiologyUnknownGenetic predisposition (↓complement =

C 1 q r s ; C2;C4 . HLA-DR2, HLA-DR3 + HLA-B8 )

Environmental = UV Light,Female Gender,EBV infection

Pathophysiology Gene-Environment interaction - Abnormal

Immune Response Auto-Antibodies (Deposition of Ig at Dermal-Epidermal Junction) Complement Activation + Inflammation (Dominated by T Lymphocytes) Irreversible Organ Damage

EpidemiologyPrevalence: 4 – 250/100 000Onset: before 8 yrs unusualFemale predominance (Child Bearing Age) (prepubertal 4:1 , postpubertal 8:1)

Butterfly Rash

Fig 65-10Fig 65-10

Diagnostic criteriaImmunologic Disorder ( Anti-dsDNA, anti Sm,

ANA )American College of Rheumatology4/11 criteria (sens 85%, specif 95% ) Malar rash – cheeks + nasal bridge Discoid rash – Erythematous,rimmed with scaling,

follicular plugging Photosensitivity Oral ulcers – painless, esp palate Arthritis – non-erosive

Diagnostic criteria continuedSerositis – Pleuritis,PericarditisRenal involvement – Proteinuria ( > 0.5

gms/day or >3+ dipstick proteinuria) ± Cellular Casts

Neurological disorders – seizures/ psychosisBlood disorders - ↓RBC,HB, PLT, WBC,

LymphocytesImmunologic Phenomena – anti-dsDNA Ab,

anti-Sm Ab, antiphospholipid AbANA – titer > 1:160

Laboratory studies High clinical suspicion/ high ANA titresSLE Screen:1. CBC2. S-creatinine3. Urinalysis with microscopy4. Basic inflammatory markers5. Antibodies to dsDNA6. Complement 7. ANA subtypes (anti-Sm, Ro, La, RNP Ab’s)8. Renal biopsy – prognosis and Rx

Autoantibody tests used in SLEANA – screening test (95% sensitivity)Anti-dsDNA (high specifcity, sens 70%)Anti-Sm (most specific Ab for SLE, 30% sens)Anti-Ro/anti-LaAnti-ribosomal PAnti-RNPAnticardiolipin (antiphospholipid Ab syndrome)Lupus Anticoagulant (antiphospholipid Ab

syndrome)Coombs test (Ab on RBC’s)Anti-histone (drug-induced lupus)

Lupus nephritis

Class IClass I Minimal mesangialMinimal mesangial Normal light microscopy; Normal light microscopy; abnormal electron microscopyabnormal electron microscopy

Class IIClass II Mesangial Mesangial proliferativeproliferative

Hypercellular on light Hypercellular on light microscopymicroscopy

Class IIIClass III Focal proliferativeFocal proliferative <50% glomeruli involved<50% glomeruli involved

Class IVClass IV Diffuse proliferativeDiffuse proliferative >50% glomeruli involved; >50% glomeruli involved; segmental/globalsegmental/global

Class VClass V MembranousMembranous Predominantly nephrotic Predominantly nephrotic diseasedisease

Class VIClass VI Advanced Advanced sclerosingsclerosing

Chronic lesions and sclerosisChronic lesions and sclerosis

Treatment principlesDepends on disease severityFever, skin, musculoskeletal and serositis =

milder diseaseCNS and Renal involvement (Class III n above

Lupus Nephrits) – Aggressive RxEmergencies: - severe CNS involvement

- systemic vasculitis - profound thrombocytopenia (TTP-like syndrome) - rapidly progressive nephritis - diffuse alveolar hemorrhage

Medications usedNSAIDSChloroquineSteroidsCyclophosphamideAzathioprineMycophenolate MofetilRituximabPlasma exchange/ IVIG

Preventive careMedication-related (steroid) complications

(Ca, vit D, bisphosphonates)Aggressive BP and lipid control Immunization (complement deficient)Avoid UV exposureAvoid estrogen therapiesAvoid sulfa-containing medicationsPregnancy planning

PrognosisBenign to rapidly progressiveBetter for isolated skin + musculoskeletal disease

vs renal and CNSDeath rate 3X age-comparable general population

Mortality

Nephritis (most within 5 yrs of symptoms) Infectious (active SLE + Rx – most common)CVS disease (50X more MI than other woman)Malignancy (chronic inflammation + Rx)

SummaryAutoimmune disorderOccurs mainly in Young WomanMultiple manifestations (The Great Imitator) Rash, Joint Symptoms(> 90%

patients),Haematological AbnormalitiesCNS and Renal Involvement – Major Source

of Disease MorbidityAggressive investigation and treatmentContinued surveillance

Beautiful BabeLook beyond n let the

Butterflies in your stomach,travel your Brains

Take Home Message Babe + Butterfly = ? SLE

Thank You !