Clinical heterogeneity in childhood-onset systemic lupus erythematosus: Single tertiary center experience Çocukluk çağı başlangıçlı sistemik lupus eritematozusta klinik heterojenite: Tek üçüncü basamak merkez deneyimi

Ertem Ş., Özçelik E., Torun Ş. E., Tekin Z. E., Öztürk D., Ekici M. I., ...Daha Fazla

Journal of Turkish Society For Rheumatology, cilt.18, sa.1, ss.75-81, 2026 (Scopus, TRDizin)

Özet

Objective: To characterize the clinical spectrum, pattern of organ involvement, treatment strategies, and disease severity in patients with childhood-onset systemic lupus erythematosus (cSLE) at a tertiary pediatric rheumatology center. Methods: This retrospective study included patients <18 years with cSLE diagnosed by Systemic Lupus International Collaborative Clinics criteria and followed between August 2019 and September 2025. Disease activity was assessed using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), and lupus nephritis was classified according to International Society of Nephrology/Renal Pathology Society criteria. Results: The age at diagnosis was 13 years [interquartile range (IQR): 11-15]. Mucocutaneous manifestations predominated, with a cutaneous rash present in 29 patients (54.7%). Other common features included non-erosive arthritis (43.4%, n=23), oral ulcers (13.2%, n=7), and alopecia (11.3%, n=6). Renal involvement occurred in 31 patients (58.5%). Renal biopsies were conducted in 25 patients (47.1% of the study population), all of whom had histopathological confirmation of lupus nephritis. The distribution among biopsied patients was as follows: Class II: 11 (20.7%); Class IV: 11 (20.7%); Class I: 2 (3.7%); and Class V: 1 (1.8%). All patients received hydroxychloroquine. Systemic corticosteroids were administered to 50 patients (94.3%). Mycophenolate mofetil was the primary immunosuppressive treatment in 43.4% (n=23) of patients, whereas cyclophosphamide was the primary immunosuppressive treatment in 41.5% (n=22) of patients. Disease activity was high at baseline (median SLEDAI-2K score: 16, IQR: 8-20), but showed a significant improvement by the final assessment (median SLEDAI-2K score: 1.5, IQR: 0-4; p<0.001).