Esteves Gladys Cherres Xavier, Gormezano Natali Weniger Spelling, Pereira Oriany L, Kern David, Silva Clovis Almeida, Pereira Rosa Maria Rodrigues, Kozu Katia Tomie, Bonfá Eloisa, Aikawa Nadia Emi
a Division of Rheumatology , Faculdade de Medicina da Universidade de São Paulo (USP) , São Paulo , SP , Brazil.
b Pediatric Rheumatology Unit , Faculdade de Medicina da Universidade de São Paulo (USP) , São Paulo , SP , Brazil.
Mod Rheumatol. 2018 Jul;28(4):649-653. doi: 10.1080/14397595.2017.1386836. Epub 2017 Oct 25.
To compare clinical and laboratorial features between childhood-onset systemic lupus erythematosus (cSLE) and adult SLE (aSLE) at concomitant diagnosis of immune thrombocytopenic purpura (ITP).
This study evaluated 56 cSLE and 73 aSLE patients regularly followed at Pediatric and Rheumatology Divisions of the same University hospital with ITP (platelets count <100,000/mm in the absence of other causes) at lupus onset.
Median current age was 11.6 and 27.3 years in cSLE and aSLE, respectively. cSLE had a higher frequency of ITP compared to aSLE (17% vs. 4%, p < .0001) and the former group had more hemorrhagic manifestations (36% vs. 16%, p = .0143). Constitutional symptoms and reticuloendothelial manifestations (p < .05), as well as pericarditis (25% vs. 10%, p = .029) and central nervous system (CNS) involvement (30% vs. 14%, p = .029) were more common in cSLE. Conversely, in aSLE, ITP was solely associated with cutaneous and articular involvements (p < .05). Concerning treatment, intravenous methylprednisolone, intravenous immunoglobulin, blood transfusion and platelets transfusion were more frequently used in the cSLE population (p < .05).
ITP at cSLE has distinct features compared to aSLE with a more severe presentation characterized by concomitant constitutional/reticuloendothelial manifestations, CNS involvement and hemorrhagic manifestation. These findings reinforce the need for a more aggressive treatment in this age group.
比较儿童期起病的系统性红斑狼疮（cSLE）和成人系统性红斑狼疮（aSLE）在并发免疫性血小板减少性紫癜（ITP）时的临床和实验室特征。
本研究评估了同一大学医院儿科和风湿病科定期随访的56例cSLE患者和73例aSLE患者，这些患者在狼疮发病时并发ITP（血小板计数<100,000/mm³且无其他病因）。
cSLE和aSLE患者的当前中位年龄分别为11.6岁和27.3岁。与aSLE相比，cSLE并发ITP的频率更高（17%对4%，p<0.0001），且前一组有更多出血表现（36%对16%，p = 0.0143）。cSLE中全身症状和网状内皮系统表现（p<0.05）以及心包炎（25%对10%，p = 0.029）和中枢神经系统（CNS）受累（30%对14%，p = 0.029）更为常见。相反，在aSLE中，ITP仅与皮肤和关节受累相关（p<0.05）。关于治疗，cSLE患者更频繁地使用静脉注射甲泼尼龙、静脉注射免疫球蛋白、输血和血小板输注（p<0.05）。
与aSLE相比，cSLE并发的ITP具有不同特征，表现更为严重，伴有全身/网状内皮系统表现、CNS受累和出血表现。这些发现强化了对该年龄组进行更积极治疗的必要性。
