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Hematological involvement in juvenile-onset systemic lupus erythematosus: Clinical features and its impact on disease
Emine Özçelik1, Elif Çelikel1, Zahide Ekici Tekin1
1Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Türkiye.
Insights
Hematological involvement affects over half of juvenile-onset systemic lupus erythematosus (jSLE) patients, often presenting with anemia or cytopenias. Treatment requires individualized approaches, especially when major organ involvement is present.
Area of Science:
- Pediatric Rheumatology
- Hematology
- Immunology
Background:
- Juvenile-onset systemic lupus erythematosus (jSLE) frequently involves hematological manifestations.
- While other organ systems often dictate initial treatment, hematological findings significantly impact patient morbidity and mortality.
- Understanding these hematological aspects is crucial for comprehensive jSLE management.
Purpose of the Study:
- To investigate the clinical characteristics of hematological involvement in jSLE patients.
- To evaluate the treatment strategies employed for jSLE patients with hematological issues.
- To identify factors associated with hematological involvement in jSLE.
Main Methods:
- Retrospective, single-center cohort study of jSLE patients diagnosed between January 2015 and May 2025.
- Inclusion based on 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria.
- Analysis of clinical data, hematological parameters, and treatment approaches.
Main Results:
- Hematological involvement was present in 52.8% of 53 jSLE patients, predominantly females (90.6%).
- Common findings included anemia (64.3%), lymphopenia (57.1%), and thrombocytopenia (50.0%).
- Constitutional symptoms, hypocomplementemia, and direct Coombs positivity were significantly more frequent in patients with hematological involvement (p=0.003, p=0.034, p=0.039). Intravenous immunoglobulin (IVIG) use was also higher (p=0.010).
Conclusions:
- Hematological involvement is a frequent complication in jSLE, observed in about half of patients.
- Hypocomplementemia and Coombs positivity are more common in jSLE with hematological issues.
- Treatment must be individualized, considering both hematological status and major organ involvement, with IVIG being a more frequent therapeutic option.
Abstract:
ObjectiveHematological involvement is a common manifestation of juvenile-onset systemic lupus erythematosus (jSLE). While nephrological and neurological involvement often guide treatment decisions in the early disease course, hematological findings may also impact morbidity and mortality. The aim of this study is to evaluate the clinical characteristics and treatment approaches of jSLE patients with hematological involvement.MethodThis retrospective, single-center cohort study was conducted on patients diagnosed with jSLE who were followed up at the pediatric rheumatology clinic between January 2015 and May 2025. Patients included in the study had been diagnosed with jSLE according to the 2012 Systemic Lupus International Collaborating Clinics classification criteria.ResultsThe study included 53 SLE patients, 48 of whom (90.6%) were female. The median age at diagnosis was 13 years (IQR: 11-15), and the median follow-up period was 26 months (IQR: 12-48). Hematological involvement was detected in 28 patients (52.8%). Anemia was observed in 18 (64.3%) of 28 patients, followed by lymphopenia in 16 (57.1%), thrombocytopenia in 14 (50.0%), and pancytopenia in 4 (14.3%). The median time to improvement of cytopenia following treatment was 32 days (IQR: 27-61). In 11 patients, treatment was based on isolated hematological findings, whereas in 17 patients with additional major organ involvement, treatment was mainly directed by the major organ manifestations. The frequency of constitutional symptoms, hypocomplementemia, and direct Coombs positivity were found to be significantly higher in patients with hematological involvement (p = 0.003, p = 0.034, p = 0.039, respectively). Intravenous ımmunoglobulin (IVIG) was also found to be administered more frequently in patients with hematological involvement (p = 0.010).ConclusionHematological involvement was detected in approximately half of jSLE patients. Hypocomplementemia and positive Coombs test were more frequently observed in patients with hematological involvement. The use of IVIG was also more common in patients with hematological involvement, and individualized treatment options remain important in the management of the disease.
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