Objective: To describe the diversity in presenting manifestations of system
ic lupus erythematosus (SLE) in children.
Study design: Initial clinical and laboratory manifestations of 39 children
, who fulfilled greater than or equal to 4 American College of Rheumatology
criteria for SLE, were retrospectively analyzed.
Results: Median age at onset was 12 years. The male to female ratio was 1:1
8.5, and racial/ethnic backgrounds were white 41%, black 33%, and Hispanic
26%. Initial manifestations included musculoskeletal 74%, cutaneous 72%, co
nstitutional 67%, neurologic 28%, renal 28%, lymphadenopathy 15%, and Rayna
ud's phenomenon 10%. Laboratory abnormalities at presentation to our clinic
included elevated erythrocyte sedimentation rate 87%, anemia 72%, lymphope
nia 59%, leukopenia 31%, proteinuria or cellular casts 44%, low C-3 or C-4
level 77%, antinuclear antibodies 97%, and anti-double-stranded DNA 95%. On
e third (33%) presented with features not initially suggestive of SLE. Six
patients presented with unusual manifestations including parotitis, quadrip
legia, chorea, severe abdominal pain, persistent cough, and dizziness. Howe
ver, 85% of patients with atypical manifestations had abnormal complete blo
od count or urinalysis results at presentation.
Conclusion: Presenting manifestations of SLE in children are diverse. A det
ailed history, thorough review of systems, complete physical examination, c
omplete blood count, urinalysis, and a high index of suspicion help to make
the correct diagnosis of SLE in patients with atypical presentations.