SYSTEMIC LUPUS ERYTHEMATOSUS- Pedia

DEFINITION Systemic Lupus International Collaborating Clinics (SLICC)

• A chronic autoimmune disease characterized by multisystem Classification Criteria for Systemic Lupus Erythematosus
inflammation and presence of circulating autoantibodies directed 4 criteria (including at least 1 clinical and 1 immunologic criterion)
against self- Ag. CLINICAL CRITEIA
Acute cutaneous Malar rash, bullous lupus, toxic epidermal
EPIDEMIOLOGY lupus necrolysis variant of SLE, maculopapular lupus
• Children, adolescents and adult rash, photosensitive lupus rash, or subacute
• More common: Female, 31 per 100,000 Asians cutaneous lupus
• Usually diagnosed in adolescents Chronic cutaneous Classic discoid rash, lupus panniculitis, mucosal
• Medial age: 11-12 y/o lupus lupus, lupus erythematous tumidus, chilblains lupus,
discoid lupus/lichen planus overlap
ETIOLOGY Oral or nasal
• Genetic abnormalities ulcers
• Estrogen exposure- promotes B-cell autoreactivity Nonscarring Diffuse thinning or hair fragility with broken hairs
• Environmental exposures- viral (EBV), ultraviolet light alopecia
Synovitis ≥2 joints, swelling, effusion
PATHOLOGY Tenderness in ≥2 joints > 30 mins of morning
• Affects: skin, joints, kidneys, blood-forming cells, blood vessels, stiffness
and CNS Serositis Pleurisy or pericardial pain ≥1 day, pleural effusion
• Discoid rash: hyperkeratosis, follicular plugging, and infiltration or rub, pericardial effusion or rub, ECG evidence of
of mononuclear cells into the dermal-epidermal junction. pericarditis
Renal Presence of red blood cell casts or urine
PATHOGENESIS protein/creatinine ratio representing >500 mg
• Hallmark: generation of autoantibodies directed against self- protein/24 hours
antigens resulting in inflammatory damage to organs Neurologic Seizures, psychosis, mononeuritis multiplex,
myelitis, peripheral or cranial neuropathy, or acute
SALIENT FEATURES confusional state
• Most common: fever, fatigue, hematologic abnormalities, Hemolytic anemia
arthralgia, and arthritis (polyarthritis- large and small joints) Leukopenia (<4,000/mm3) or lymphopenia (<1,000/mm3)
• Renal disease: edema, fatigue, changes in urine color, and Thrombocytopenia (<100,000/mm3)
nausea/vomiting IMMUNOLOGIC CRITERIA
Positive antinuclear antibody
DIFFERENTIAL DIAGNOSIS Positive double-stranded DNA antibody
• Infections- sepsis, Epstein-Barr virus, parvovirus B19, endocarditis Positive anti-Smith antibody
• Malignancies- leukemia and lymphoma Antiphospholipid antibody positivity
• Poststreptococcal glomerulonephritis, other rheumatologic Low complement- Low C3, C4, or Ch50 level
conditions- systemic onset juvenile idiopathic arthritis, vasculitides Positive direct Coombs test
• Drug-induced lupus- minocycline, anticonvulsants, sulfonamides,
antiarrhythmic agents Mnemonic
S erositis B lood abnormalities M alar rash
DIAGNOSTICS O ral ulcers R enal D iscoid rash
A rthritis A NA
American College of Rheumatology (ACR) 1997 Revised P hotosensitivity I mmunologic
Classification Criteria- 4 of 11 criteria establishes the diagnosis of SLE N eurologic manifestations
CLINICAL CRITEIA
Malar rash Fixed erythema, flat or raised over the malar MANAGEMENT
eminence, spare nasolabial folds
Discoid rash Erythematous raised patches w/ adherent MILD DISEASE MODERATE- SEVERE
keratotic scaling Patient who does not have renal or With significant organ involvement
Photosensitivity Rash from unusual exposure to sunlight other life-threatening involvement and life-threatening situation
Oral or nasal ulcers Usually painless • NSAIDS • High dose glucocorticoids
Arthritis Nonerosive arthritis, > 2 peripheral joints. • Hydroxychloroquine • Hydroxychloroquine
Tender, swelling, effusion Low dose glucocorticoids • Mycophenolate mofetil
Serositis Pleuritis OR • Cyclophosphamide
Pericarditis (ECG), rub, effusion • Rituximab
Renal manifestations Proteinuria >500 mg/24h or >3+ OR If persistent: Methotrexate,
Cellular cast Leflunomide, Azathioprine
Seizure or psychosis
Hematologic Hemolytic anemia w/ reticulocytosis OR PROGNOSIS
manifestations Leukopenia <4000/mm3 on > occasions OR • 5 yr survival rate for pediatric SLE is ~95%, though the 10 yr
lymphopenia <1500/ mm3 on > occasions OR survival rate remains ~80-90%
thrombocytopenia <100,000/ mm3
IMMUNOLOGIC CRITERIA
Immunologic Anti- DNA or Anti- Smith antibody or
abnormalities Antiphospholipid antibody
Positive antinuclear
antibody test result