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systemic lupus erythematosus (SLE) - multisystem disorder that is characterized by
widespread inflammatory involvement of connective tissues with immune complex vasculitis
SLE Etiology/Incidence - unknown, but many factors implicated: Excessive sun exposure,
drug reaction, infection, hereditary, immunogenetic.
SLE can develop at any age - usually after 5 years, more common during adolescent years
in females.
Females affected eight times more often after 5 years of age
SLE signs and symptoms - 1. fever
2. malaise
3. Weight loss
4. Malar facial rash
5. Arthralgias
SLE: Differential Diagnosis - Juvenile rheumatoid arthritis (JRA)
Other forms of acute glomerulonephritis
Hemolytic anemia
Leukemia
Allergic or contact dermatitis
Idiopathic seizure disorder
Mononucleosis
Acute rheumatic fever with carditis
Septicemia
Toxic exposure
, SLE: Physical findings 1-5 - 1. Onset is usually acute--three quarters of children usually
diagnosed within 6 months of symptoms
2. Diagnosis delayed for others by 4-5 years
3. Early diagnostic suspicion based on:
a. Episodic, multisystem constellation of clinical disease
b. Associated with persistent antinuclear antibody (ANA) seropositivity
4. Severity of manifestations variable
a. Rapidly fatal illness
b. malar erythematous
5. Each exacerbation of disease tends to mimic previous episodes
SLE: Physical Findings 6-10 - 6. Rash
a. Characteristic of acute onset or exacerbation
b. malar erythematous
c. butterfly distribution across bridge of nose and over each cheek
d. discoid rash over sun-exposed areas
7. Arthritis
a. Affects majority of children
b. Involves small joints
c. Transient and migratory
d. never erosive
e. possible avascular necrosis of bone in 25% of children
8. Pericarditis is most common manifestation of cardiac involvement--murmurs associated with
endocarditis
9. Central and peripheral nervous system manifestations
a. recurrent headaches
b. seizures