1. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of auto-antibodies against components of the cell nucleus. 2. SLE affects multiple organ systems and is more common in females, with a female to male ratio of 9:1 before puberty. 3. Diagnosis of SLE requires meeting 4 out of 11 American College of Rheumatology diagnostic criteria, including at least 1 clinical and 1 immunological criterion. Common clinical manifestations include malar rash, arthritis, renal disease, and hematological abnormalities.

1. Pediatric
Systemic Lupus
Erythematosus
Dr.C.S.N.Vittal
Associate Professor of Pediatrics
ASRAM

2.  SLE has been classically described as a prototypic
autoimmune disease with a wide array of clinical
manifestations and characterized by the production
of auto-antibodies to components of the cell
nucleus.
 Gilbert et al. Pediatric Rheumatology 2014, 12:16
Systemic Lupus Erythematosus

3. z
SLE - Historical
Classical
Neoclassical
Modern
First description of the cutaneous disorder;
term "lupus" was coined. "lupus" that means wolf in
Latin. 13th Century physician Rogerius
1872: Moriz Kaposi : description of the disease’s
systemic or disseminated manifestations,
two forms of the disease — what we know as systemic
lupus erythematosus and discoid lupus
Discovery of the LE cell in bone marrow in 1948 by
Malcolm Hargraves
1957 : first anti-DNA antibody

4. z
Female:Male = 9:1, but before puberty it is 3:15
pSLE makes 20% of all cases1
Incidence <15 yrs: 0.5-0.6 per 100,000 persons2
Prevalence 4 – 250 cases per 100, 000 persons3
More than 90% cases of SLE occur in women4
SLE - Epidemiology

5. z SLE - ETIOPATHOGENY
IRF 5,

6. z
S
L
E

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SLE Pathology
 The LE cell is a neutrophil that has
engulfed the antibody-coated nucleus of
another neutrophil.
 LE cells may appear in rosettes where
there are several neutrophils vying for an
individual complement covered protein.
• Immune complexes within the dermal–
epidermal junction.
• “lupus band test” which is specific for SLE.

8. z

9. 1. Malar rash
2. Naso-oral ulcers
3. Photosensitive rash
4. Discoid rash
5. Arthritis
6. Pleuritis or pericarditis
7. Proteinuria (>500 mg/d) or evidence of nephritis in urinalysis
8. Hemolytic anemia, thrombocytopenia, leukopenia, or lymphopenia
9. Seizure or psychosis
10. Positive ANA finding
11. IMMUNOLOGIC ABNORMALITIES: Positive anti–dsDNA, anti-Smith, or
antiphospholipid antibody / lupus anticoagulant
The Systemic Lupus International Collaborative Clinics (SLICC) recently published a modification of
the ACR criteria.
Lupus patients meet 4 criteria with at least 1 clinical and 1 immunologic criterion or
with biopsy-proven nephritis in association with positive ANA and anti-dsDNA
The ACR’s ( American College of Rheumathology) diagnostic criteria for SLE :

10. 1. Serositis
2. Oral ulcers
3. Arthritis
4. Photosensitivity
5. Blood disorders
6. Renal manifestations
7. ANAs
8. Immunological phenomena
9. Neurological disorder
10. Malar rash
11. Discoid rash
ACR mnemonic of SLE diagnostic criteria ----"SOAP BRAIN MD"

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Systemic lupus erythematosus (SLE), most common form (70%)
Cutaneous lupus erythematosus (CLE) – (about 10%)
Drug-induced lupus
Neonatal lupus
Lupus – Types

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SLE – Clinical
Mucocutaneous Manifestations: 76% Joint Problems:
• Malar rash - butterfly-like redness
• Discoid lupus
• Vasculitis (purpura, petechiae)
• Raynaud’s phenomenon
• Nail involvement
• Alopecia
• Periungual erythema/ Livedo
reticularis
• Photosensitivity
• Oral/ nasal ulcers
• Intermittent arthralgias to
sudden inflammation of
multiple joints occur in about
90% of people
• In long-standing disease,
marked joint deformity may
occur (Jaccoud's
arthropathy) but is rare.
• Symptoms may remain

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Malar rash - butterfly-like redness

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Vasculitis in SLE

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Discoid rash

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SLE – Clinical
Pulmonary Findings
(5-67%)
Cardiovascular
Findings
• May be subclinical
(abnormal PFTs)
• Pleuritis
• Pleural effusion
• Pneumonitis
• Pulmonary
hemorrhage
• Pulmonary
hypertension
• Restrictive lung
disease
• Pericarditis
• Myocarditis
• Sterile valvular
vegetations
(Libman Sach’s)
• Arrhythmias
• Cor pulmonale
• Vasculitis
• Atherosclerosis /
Coronary Heart
disease
• Dyslipoproteinemias

20. z
SLE – Clinical
Neuropsychiatric Manifestations (20-40% )
• Second to nephritis as most common cause of
morbidity & mortality
• Can occur at any time; even at presentation
COMMON • Depression, organic brain
syndrome, functional psychosis,
headaches, seizures, cognitive
impairment, dementia, coma
OCCASIONAL • Cerebral vascular accidents,
aseptic meningitis, peripheral
neuropathy, cranial nerve palsies
RARE: • Paralysis, transverse myelopathy,
chore

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SLE – Clinical
Hematologic Findings
• Leukopenia, especially lymphopenia
• Anemia
• mild to moderate, due to chronic disease and mild hemolysis
• severe, uncommon (5%), due to immune mediated hemolysis
(Coombs +)
• Thrombocytopenia
• Bone marrow suppression / arrest -- very rare
• Coagulopathy – prolonged PTT

22. z
SLE – Clinical
Renal Findings
Most common cause of morbidity & mortality
• Glomerulonephritis – at least 75%
• Microscopic or gross hematuria
• Proteinuria, including nephrotic syndrome
• Hypertension
• Decreased GFR
• Renal failure (up to 30-50% of children prior to 1980)
• Renal biopsy predictive of potential for renal damage

23. z
International Society of Nephrology 2003 Revised Classification of SLE Nephritis
Class Classification Features
Class I
Minimal mesangial Normal light microscopy findings; abnormal electron
microscopy findings
Class II
Mesangial
proliferative
Hypercellular on light microscopy
Class III Focal proliferative < 50% of glomeruli involved
Class IV Diffuse proliferative
=50% of glomeruli involved; classified segmental or
global;
to be treated aggressively
Class V Membranous
Predominantly nephrotic disease may occur with
class III or IV
Class VI Advanced
sclerosing
=90% of glomeruli involved without residual activity
Chronic lesions and sclerosis

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25. z
NeonatalLupus
• Passive transplacental transfer of maternal
anti-Ro/SSA and anti-La/ SSB antibodies
causes the transient skin lesions
• Characteristic annular or macular rash typically
affecting the face, trunk & scalp – usually clear
by 6 mo age
• Ultraviolet light may exacerbate or initiate
cutaneous lesions.
• Cytopenias and
• Cholestatic liver disease
• Congenital heart block (30%)- most feared
complication
Annular plaque

26. z
SLE - Laboratory Findings
 Cytopenias (anemia, thrombocytopenia,
leukopenia)
 Elevated ESR, CRP, Immunoglobulins
 Hypoalbuminemia
 Proteinuria; RBCs, casts in urine
 Decreased creatinine clearance
 Low complement levels (C3/ C4)
 Autoantibodies

27. z
Auto-antibodies
 ANA - Sensitive but not specific, 95-98% pts positive
Against nuclear components of the cell
 Other :
 Anti dsDNA : high specificity for lupus (over 80%)
 RNP assoc w/ MCTD (Mixed connective-tissue disease),
 Anti Smith Abs : specific for SLE
 Ro/ La (SS-a/ SS-b): neonatal lupus, Sjogren’s
 Anti Histone Abs : drug induced lupus

28. z
SLE - Treatment
 MILD DISEASE: Rashes, arthralgias, leukopenia,
anemia, arthritis, fever, fatigue
 Treatment:
 NSAIDs
 low dose corticosteroids (< 60 mg/day)
 antimalarials (hydroxychloroquine)

29. z
SLE - Treatment
 MODERATE DISEASE: MILD + OTHER SYSTEM
INVOLVEMENTS
 Treatment:
 Prednisone 1-2 mg/kg/day,
 NSAIDS,
 Antimalarials,
 Azathioprine

30. z
SLE - Treatment
 SEVERE DISEASE: Severe, life-threatening organ system
involvement (e.g. Class IV lupus nephritis, myocarditis,
encephalopathy)
 High dose corticosteroids (2-3 mg/kg/day)
 Immunosuppressives:
 IV pulse methyl prednisolone / dexamethasone
OR
 Monthly pulses of Cyclophosphamide),
 Plasmapheresis,
 Anticoagulation where appropriate

31. z
Management of Pediatric SLE
 General Use high-SPF sunscreen throughout the year.
 Encourage good sleep and nutritional patterns.
 Address psychological aspects of disease/treatment.
 Prescribe calcium and vitamin D supplements
 Immunize against pneumococcus.
 Anticoagulant if evidence of antiphospholipid antibody is
present
 Perform annual ophthalmologic evaluations
 Treat dyslipoproteinemia when present.
 Maintain good blood pressure control in those with
hypertension

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Summary

33. z
Dr C.S.N.Vittal