Diversity in presenting manifestations of systemic lupus erythematosus in children,☆☆

https://doi.org/10.1016/S0022-3476(99)70174-5Get rights and content

Abstract

Objective: To describe the diversity in presenting manifestations of systemic lupus erythematosus (SLE) in children. Study design: Initial clinical and laboratory manifestations of 39 children, who fulfilled ≥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 1:18.5, and racial/ethnic backgrounds were white 41%, black 33%, and Hispanic 26%. Initial manifestations included musculoskeletal 74%, cutaneous 72%, constitutional 67%, neurologic 28%, renal 28%, lymphadenopathy 15%, and Raynaud’s phenomenon 10%. Laboratory abnormalities at presentation to our clinic included elevated erythrocyte sedimentation rate 87%, anemia 72%, lymphopenia 59%, leukopenia 31%, proteinuria or cellular casts 44%, low C3 or C4 level 77%, antinuclear antibodies 97%, and anti-double-stranded DNA 95%. One third (33%) presented with features not initially suggestive of SLE. Six patients presented with unusual manifestations including parotitis, quadriplegia, chorea, severe abdominal pain, persistent cough, and dizziness. However, 85% of patients with atypical manifestations had abnormal complete blood count or urinalysis results at presentation. Conclusion: Presenting manifestations of SLE in children are diverse. A detailed history, thorough review of systems, complete physical examination, complete blood count, urinalysis, and a high index of suspicion help to make the correct diagnosis of SLE in patients with atypical presentations. (J Pediatr 1999;135:500-5)

Section snippets

PATIENTS AND METHODS

All children with SLE who were seen in our tertiary care pediatric rheumatology clinic between 1991 and 1998 were studied. This group also included those patients with SLE who were initially referred to the pediatric nephrology service at All Children’s Hospital in St Petersburg, Florida. All of the 39 patients reported in this study fulfilled ≥4 American College of Rheumatology criteria for the diagnosis of SLE.14, 15 Patients with drug-induced lupus or mixed connective tissue disease were

RESULTS

The age at onset of SLE among the 39 children who met entry criteria ranged from 5 to 18 years. Fifty-four percent of children presented between the ages of 10 and 14 years. The median age at onset was 12 years. The male to female ratio was 1:18.5. Of the 39 patients, 16 were white (41%), 13 were black (33%), and 10 were Hispanic (26%). In our catchment area 89% of the general population is white.

DISCUSSION

When SLE presents with atypical initial manifestations such as cough, abdominal pain, or parotitis, there is often a delay in the diagnosis. Although SLE is mostly observed in women aged 20 to 40 years, onset in approximately 15% of cases is seen in children younger than 16 years.3, 5, 6, 7, 8, 9, 10, 11, 12 Results of our study are comparable with most previous pediatric series of SLE.3, 11, 16, 17, 18, 19, 20, 21 Clinical manifestations of SLE in children generally resemble those in adults.

Acknowledgements

We thank Dr N. K. Day, Director of the Immunoparameters Laboratory at the All Children’s Hospital, St Petersburg, Florida, for her valuable contribution. We also thank Ms Tazim Verjee for manuscript preparation.

References (37)

  • AG Meislin et al.

    Systemic lupus erythematosus in childhood

    Pediatrics

    (1968)
  • TJA Lehman et al.

    Systemic lupus erythematosus in the first decade of life

    Pediatrics

    (1989)
  • JL Platt et al.

    Systemic lupus erythematosus in the first two decades of life

    Am J Kidney Dis

    (1982)
  • JT Cassidy et al.

    Lupus nephritis and encephalopathy: prognosis in 58 children

    Arthritis Rheum

    (1977)
  • PA Walravens et al.

    The prognosis of childhood systemic lupus erythematosus

    Am J Dis Child

    (1976)
  • CL Yancey et al.

    Central nervous system involvement in childhood systemic lupus erythematosus

    Arthritis Rheum

    (1981)
  • R Cervera et al.

    Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on SLE

    Medicine

    (1993)
  • EM Tan et al.

    The 1982 revised criteria for the classification of systemic lupus erythematosus

    Arthritis Rheum

    (1982)
  • Cited by (0)

    Reprint requests: Shahid Iqbal, MD, All Children’s Hospital, 801 Sixth St South, St Petersburg, Florida 33701.

    ☆☆

    0022-3476/99/$8.00 + 0  9/21/101553

    View full text