Hematological involvement in juvenile-onset systemic lupus erythematosus: Clinical features and its impact on disease management
Emine Özçelik, Elif Çelikel, Zahide Ekici Tekin, Şeyma Ertem, Merve Cansu Polat, Şeyma Erdem Torun, Didem Öztürk, Mehveş Işıklar Ekici, Yasemin Uğur Es, Sultan Nilay Yoğun, Cüneyt Karagöl, Özlem Arman Bilir, Neşe Yaralı, Namık Yaşar Özbek, Banu Çelikel AcarObjective
Hematological 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.
Method
This 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.
Results
The 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 (
Conclusion
Hematological 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.