Severe haematological involvement in children with systemic lupus erythematosus and clinical associations

Kisaoglu, Hakan; Sener, Seher; Demirbas, Kaan; Yigit, Yasemin; Garipcin, Pinar; Coşkun, Serkan; Kacmaz, Hatice; Ekinci, Rabia; Ozturk, Kubra; Koker, Oya; Ucak, Kubra; Tuncez, Serife; Kilbas, Gulsah; Karacayir, Nihal; Baykal, Gulcan; Taskın, Sema; Bozkaya, Burcu; Baba, Ozge; DEMİR, SELCAN; BAŞARAN, HALİDE; Sahin, Sezgin; Baglan, Esra; ŞAHİN, NİHAL; GÜNGÖRER, Vildan; Kısaarslan, Aysenur; SÖZERİ, Betül; YÜKSEL, SELÇUK; Bakkaloglu, Sevcan; Balat, Ayse; Gurgoze, Metin; KASAPÇOPUR, ÖZGÜR; Ozen, Seza; KALYONCU, MUKADDES

doi:10.1093/rheumatology/keae414

Severe haematological involvement in children with systemic lupus erythematosus and clinical associations

Kisaoglu H., Sener S., Demirbas K. C., Yigit Y. D., Garipcin P., Coşkun S., ...Daha Fazla

Rheumatology, cilt.64, sa.4, ss.2153-2161, 2025 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 64 Sayı: 4
Basım Tarihi: 2025
Doi Numarası: 10.1093/rheumatology/keae414
Dergi Adı: Rheumatology
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, BIOSIS, CINAHL, EMBASE, MEDLINE
Sayfa Sayıları: ss.2153-2161
Anahtar Kelimeler: children, damage, flare, haematological involvement, systemic lupus erythematosus, treatment
Sağlık Bilimleri Üniversitesi Adresli: Evet

Özet

Objectives: To investigate the severe haematological involvement in children with SLE and assess its clinical associations, treatments, outcome and damage accrual. Methods: The medical charts of children with SLE in whom haematological involvement was observed were reviewed. Severe haematological indices were defined as autoimmune haemolytic anaemia with a haemoglobin concentration <8 g/dl, thrombocyte count <30 000/µL and neutrophil count <500/µL. Results: Among the 224 patients included, 102 (45.5%) displayed severe indices, predominantly at the initial involvement, and most frequently as severe anaemia in 54 (24.1%) and severe thrombocytopenia in 45 (20.1%). Disease activity did not differ according to the presence of severe disease indices. In addition, the presence of severe indices at initial involvement did not affect the damage accrual. However, a higher rate of damage (51.1% vs 29.9%, P 0.002) and steroid-induced damage (28.9% vs 8.2%, P < 0.001) was evident in patients with flares of the haematological system. Regression analysis revealed that rituximab treatment during the initial episode (OR: 4.5, P 0.006) and the presence of anticardiolipin antibodies (OR: 2.3, P 0.014) significantly increases the odds for haematological system flare. However, severe indices at initial involvement did not increase the odds of a haematological flare. Conclusion: Severe haematological indices at onset are common but not related with disease outcomes. Prevention of flares is important to improve outcomes, and a more rigorous maintenance strategy would benefit most to children who display haematological indices refractory to conventional immunosuppressants and those with anti-cardiolipin antibodies.