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https://hdl.handle.net/11499/57904
Title: | Severe haematological involvement in children with systemic lupus erythematosus and clinical associations | Authors: | Kisaoglu, Hakan Sener, Seher Demirbas, Kaan Can Demir Yigit, Yasemin Garipcin, Pinar Coskun, Serkan Kacmaz, Hatice Melisa Ekinci, Rabia Miray Kisla Ozturk, Kubra Koker, Oya Ucak, Kubra Tuncez, Serife Kilbas, Gulsah Karacayir, Nihal Baykal, Gulcan Ozomay Taskin, Sema Nur Bozkaya, Burcu Baba, Ozge Demir, Selcan Basaran, Ozge Sahin, Sezgin Baglan, Esra Sahin, Nihal Gungorer, Vildan Kisaarslan, Aysenur Pac Sozeri, Betul Yuksel, Selcuk Bakkaloglu, Sevcan Balat, Ayse Gurgoze, Metin Kaya Kasapcopur, Ozgur Ozen, Seza Kalyoncu, Mukaddes |
Keywords: | children damage flare haematological involvement systemic lupus erythematosus treatment Macrophage Activation Syndrome Disease Onset Prevalence Juvenile Cohort Damage Thrombocytopenia Validation Management |
Publisher: | Oxford Univ Press | Abstract: | 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/mu L and neutrophil count <500/L. 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. | URI: | https://doi.org/10.1093/rheumatology/keae414 https://hdl.handle.net/11499/57904 |
ISSN: | 1462-0324 1462-0332 |
Appears in Collections: | PubMed İndeksli Yayınlar Koleksiyonu / PubMed Indexed Publications Collection Tıp Fakültesi Koleksiyonu WoS İndeksli Yayınlar Koleksiyonu / WoS Indexed Publications Collection |
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