2. Patient
35 years old female
Presented 2 mths ago with right shoulder pain
Initially treated as Reactive Arthritis – Unrelieved
Admitted outside for sudden eruption of rashes
and oozing crusts over lips n face and epistaxis
with generalized anasarca
2 seizures after discharge
No history of medicines, allergies, family risk
Infertility (Laparoscopy,IVF –Unsuccessful)
k/c/o Hypothyroidism

3. On Examination
Obese
Apyrexial, RR 20, HR 110, BP 144/70
Erythematous Rash on body,Crusts n Oozing
lesions over lower lips and left ankle, Oral Ulcers
Severe Pallor, Moderate pitting pedal oedema
CVS: tachycardic
Resp, Abdo, neuro : normal

4. Laboratory investigations
Hb 6.6 gm% , Plt 96,000/cmm
TLC 2200/cmm
ESR 47
Anti-CCP Negative
INR, PTT, BT/CT : normal
S.Creat = 2.1 mg%
Urine R/M s/o Proteinuria + Haematuria

5. Laboratory investigations
Hb 6.6 gm% , Plt 96,000/cmm
TLC 2200/cmm
ESR 47
Anti-CCP Negative
INR, PTT, BT/CT : normal
S.Creat = 2.1 mg%
Urine R/M s/o Proteinuria + Haematuria
ANA,Anti-DsDNA = Positive

6. Treatment Given
IV Methyl Prednisolone
Followed by Oral Prednisolone ( 1 mg/kg/day)

7. Current Condition of Patient
Asymptomatic
Ambulatory
Shoulder Pain – Better
Pancytopenia – Reverted back to Normal counts
Renal Function – Urine – no Proteinuria,
S.Creat = 1.2 mg%

8. Introduction to SLE
Auto-immune disorder
Multisystem microvascular inflammation
Formation of autoantibodies
Chronic with relapsing and remitting course

9. Etiology
Unknown
Genetic predisposition ( complement↓ = C 1 q r s ;
C2;C4 . HLA-DR2, HLA-DR3 + HLA-B8 )
Environmental = UV Light,Female Gender,EBV
infection

10. 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

11. Epidemiology
Prevalence: 4 – 250/100 000
Onset: before 8 yrs unusual
Female predominance (Child Bearing Age)
(prepubertal 4:1 , postpubertal 8:1)

13. Butterfly Rash
Fig 65-10Fig 65-10

14. Diagnostic criteria
Immunologic Disorder ( Anti-dsDNA, anti Sm, ANA )
American College of Rheumatology
4/11 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

15. Diagnostic criteria continued
Serositis – Pleuritis,Pericarditis
Renal involvement – Proteinuria ( > 0.5 gms/day
or >3+ dipstick proteinuria) ± Cellular Casts
Neurological disorders – seizures/ psychosis
Blood disorders - RBC,HB, PLT, WBC,↓
Lymphocytes
Immunologic Phenomena – anti-dsDNA Ab, anti-
Sm Ab, antiphospholipid Ab
ANA – titer > 1:160

16. Laboratory studies
High clinical suspicion/ high ANA titres
SLE Screen:
1. CBC
2. S-creatinine
3. Urinalysis with microscopy
4. Basic inflammatory markers
5. Antibodies to dsDNA
6. Complement
7. ANA subtypes (anti-Sm, Ro, La, RNP Ab’s)
8. Renal biopsy – prognosis and Rx

17. Autoantibody tests used in SLE
ANA – screening test (95% sensitivity)
Anti-dsDNA (high specifcity, sens 70%)
Anti-Sm (most specific Ab for SLE, 30% sens)
Anti-Ro/anti-La
Anti-ribosomal P
Anti-RNP
Anticardiolipin (antiphospholipid Ab syndrome)
Lupus Anticoagulant (antiphospholipid Ab syndrome)
Coombs test (Ab on RBC’s)
Anti-histone (drug-induced lupus)

18. Lupus nephritis
Class IClass I Minimal mesangialMinimal mesangial Normal light microscopy;Normal light microscopy;
abnormal electron microscopyabnormal electron microscopy
Class IIClass II MesangialMesangial
proliferativeproliferative
Hypercellular on lightHypercellular 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 nephroticPredominantly nephrotic
diseasedisease
Class VIClass VI AdvancedAdvanced
sclerosingsclerosing
Chronic lesions and sclerosisChronic lesions and sclerosis

19. Treatment principles
Depends on disease severity
Fever, skin, musculoskeletal and serositis =
milder disease
CNS and Renal involvement (Class III n above
Lupus Nephrits) – Aggressive Rx
Emergencies: - severe CNS involvement
- systemic vasculitis
- profound thrombocytopenia
(TTP-like syndrome)
- rapidly progressive nephritis
- diffuse alveolar hemorrhage

20. Medications used
NSAIDS
Chloroquine
Steroids
Cyclophosphamide
Azathioprine
Mycophenolate Mofetil
Rituximab
Plasma exchange/ IVIG

21. Preventive care
Medication-related (steroid) complications (Ca,
vit D, bisphosphonates)
Aggressive BP and lipid control
Immunization (complement deficient)
Avoid UV exposure
Avoid estrogen therapies
Avoid sulfa-containing medications
Pregnancy planning

22. Prognosis
Benign to rapidly progressive
Better for isolated skin + musculoskeletal disease vs renal
and CNS
Death 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)

23. Summary
Autoimmune disorder
Occurs mainly in Young Woman
Multiple manifestations (The Great Imitator)
Rash, Joint Symptoms(> 90%
patients),Haematological Abnormalities
CNS and Renal Involvement – Major Source of
Disease Morbidity
Aggressive investigation and treatment
Continued surveillance

24. Beautiful
Babe
Look beyond n let
the
Butterflies
in your stomach,
travel your
Brains

25. Take Home Message
Babe + Butterfly = ? SLE

26. Thank You !