Bashyal Krishna Prasad, Shah Sangam, Ghimire Calvin, Balmuri Shravya, Chaudhary Pradip, Karki Sandip, Poudel Anuj Krishna, Pokharel Ashbina, Devarkonda Vishal, Hayat Samina
McLaren Health Care Corp, 401 South Ballenger Hwy, Flint 48532, USA.
Tribhuvan University, Institute of Medicine, Maharajgunj, Kathmandu 44600, Nepal.
Int J Rheumatol. 2024 Mar 1;2024:6650921. doi: 10.1155/2024/6650921. eCollection 2024.
Immune thrombocytopenic purpura (ITP) is an immune-mediated disorder characterized by the destruction of platelets and megakaryocytes due to autoantibodies against the platelet surface proteins. ITP without any apparent cause of thrombocytopenia is defined as primary ITP, and ITP in the setting of SLE is secondary ITP, which can be diagnosed after excluding other causes of thrombocytopenia by history, physical examination, and laboratory testing. Patients with ITP associated with SLE have higher median platelet count and less bleeding manifestations compared to the patients with primary ITP. It can be very challenging to diagnose primary ITP in SLE patients as other causes of thrombocytopenia including drug-induced thrombocytopenia, antiphospholipid syndrome, and thrombotic microangiopathic process should be ruled out. Corticosteroids are the main modality of treatment. IVIG can be used in severe cases. Splenectomy was found to be less effective in ITP associated with SLE compared to primary ITP. Control of disease activity with immunosuppressive therapy can be helpful in some cases associated with active disease flares in SLE patients.
免疫性血小板减少性紫癜（ITP）是一种免疫介导的疾病，其特征是由于针对血小板表面蛋白的自身抗体导致血小板和巨核细胞被破坏。无任何明显血小板减少病因的ITP被定义为原发性ITP，而系统性红斑狼疮（SLE）背景下的ITP为继发性ITP，通过病史、体格检查和实验室检查排除其他血小板减少病因后可确诊。与原发性ITP患者相比，合并SLE的ITP患者的血小板计数中位数更高，出血表现更少。在SLE患者中诊断原发性ITP可能极具挑战性，因为需要排除其他血小板减少病因，包括药物性血小板减少、抗磷脂综合征和血栓性微血管病过程。糖皮质激素是主要的治疗方式。严重病例可使用静脉注射免疫球蛋白（IVIG）。与原发性ITP相比，脾切除术在合并SLE的ITP中疗效较差。在一些与SLE患者疾病活动发作相关的病例中，使用免疫抑制疗法控制疾病活动可能会有帮助。
