Initial presentation of childhood-onset systemic lupus erythematosus: A French multicenter study

Objective

To describe the clinical and laboratory manifestations of childhood-onset systemic lupus erythematosus (SLE) at presentation.

Study design

This retrospective French multicenter study involved 155 patients in whom SLE developed before the age of 16 years. Mean patient age at onset was 11.5 ± 2.5 years (range, 1.5-16 years). The female to male ratio was 4.5.

Results

The most common initial manifestations were hematologic (72%), cutaneous (70%), musculoskeletal (64%), renal (50%), and fever (58%). Thirty-two percent of children had atypical symptoms, mainly including abdominal involvement in 26 patients, which lead to negative laparotomy results for presumed appendicitis. Severe renal, neurologic, hematologic, abdominal, cardiac, pulmonary, thrombotic, and/or cutaneous manifestations occurred within the first month after the diagnosis in 40% of patients. The mean erythrocyte sedimentation rate was 72 ± 29 mm/h, and the mean C-reactive protein value 22 ± 21 mg/L. Antinuclear antibodies an, anti-double stranded DNA antibodies, and low C3 or C4 level were retrieved in 97%, 93%, and 78 % of patients, respectively.

Conclusion

Initial manifestations of childhood-onset SLE are diverse and often severe. The diagnosis of SLE should be promptly considered in any febrile adolescent with unexplained organ involvement, especially when associated with an increased erythrocyte sedimentation rate.

SLE, Systemic lupus erythematosus, ANA, Antinuclear antibodies, Anti-dsDNA, Anti-double-stranded DNA, LAC, Lupus anticoagulant, WHO, World Health Organization, ACR, American College of Rheumatology, HUS, Hemolytic-uremic syndrome, WBC, White blood cell, ESR, Erythrocyte sedimentation rate, CRP, C-reactive protein