In patients with pediatric systemic lupus erythematosus (SLE), hematologic abnormalities are frequent, and hematologic involvement is associated with positive antiphospholipid (APL) antibodies and high Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores, according to study results published in Lupus.

Medical records of pediatric patients with SLE, who met the Systemic Lupus International Collaborating Clinics (SLICC) criteria, from multiple centers were retrospectively analyzed between January and June 2000.

A total of 215 children with SLE were included in the study, 118 of whom had hematologic manifestations. At the time of diagnosis, age was similar among participants, but time from symptom onset to diagnosis was longer among those without vs with hematologic involvement (4.2 vs 2.0 months, respectively; P =.013). In both groups, the most frequent SLE presentation was acute cutaneous lupus.

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In patients with hematologic involvement, renal involvement was more frequent (P =.04), as well as low C3 levels (P =.008), anti-cardiolipin (P =.001), anti-beta-2-glycoprotein I (P <.001), anti-Smith antibody (P <.001) positivity, and presence of lupus anticoagulant (P <.001). Patients with vs without hematologic involvement also had higher mean SLEDAI scores at the time of diagnosis (13.62±9.19 vs 9.61±4.58, respectively; P =.001).

At diagnosis, anemia was present in 74 (62.7%) patients, thrombocytopenia in 43 (36.4%), leukopenia in 41 (34.7%), lymphopenia in 35 (29.6%), and neutropenia in 21 (17.7%). During the follow-up period, the most frequent hematologic abnormalities were leukopenia (n=21; 17.7%), lymphopenia (n=16; 13.5%), anemia (n=14; 11.8%), thrombocytopenia (n=11; 9.3%), and neutropenia (n=11; 9.3%). The most common form of anemia was autoimmune hemolytic anemia and was diagnosed in 40 (45.4%) patients. Mean SLEDAI score at the time of hematologic involvement was 14.3±9.2.

The most common treatment agents for hematologic involvement were intravenous immunoglobulin (IVIG) and corticosteroids. For patients with an intolerant or inadequate response to corticosteroids and IVIG treatments, rituximab was preferred, followed by mycophenolate mofetil, cyclosporine, cyclophosphamide, and plasmapheresis.

Factors independently associated with hematologic involvement were the presence of APL antibodies (odds ratio [OR], 0.249; 95% CI, 0.126-0.490; P <.001) and high SLEDAI score (OR, 1.136; 95% CI, 1.065-1.212; P <.001).

Study limitations included the retrospective design and the inability to evaluate and compare the efficacy of treatment strategies used by each center.

Researchers concluded, “Controlled studies are needed to conclude on the best treatment algorithms for hematological involvement of SLE.”


Akca ÜK, Batu ED, Kisaarslan AP, et al. Hematological involvement in pediatric systemic lupus erythematosus: A multi-center study. Lupus. Published online May 6, 2021. doi:10.1177/09612033211014271

This article originally appeared on Rheumatology Advisor