结缔组织病伴免疫性血小板减少症的诊断治疗进展

Progress in the diagnosis and treatment of connective tissue disease associated with immune thrombocytopenia

免疫性血小板减少症(ITP)为免疫介导的疾病,分为原发性ITP和继发性ITP。结缔组织病(CTD)是一组主要侵犯全身结缔组织的系统性自身免疫性疾病,容易合并继发性ITP的疾病包括系统性红斑狼疮、原发性干燥综合征、抗心磷脂抗体综合征等。CTD-ITP患者出血倾向增加,严重者可致内脏出血,病死率高,是影响CTD患者预后的重要因素。近年来有关CTD-ITP的发病机制、临床表现、治疗方法的研究取得了较大进展,新型生物制剂和促血小板生成药物的使用让更多患者获益。早期诊断、出血程度分级、风险判断以及个性化达标治疗是获得良好预后的关键。

Immunological thrombocytopenia(ITP)is an immune-mediated disease,divided into primary ITP and secondary ITP.Connective tissue disease(CTD)is a group of systemic autoimmune diseases that mainly invade the whole body's connective tissue.It is the most common disease to combine with secondary ITP,including systemic lupus erythematosus,primary Sjogren syndrome,and antiphospholipid antibody syndrome.CTD-ITP patients have an increased tendency for bleeding,and in severe cases,it can lead to visceral bleeding with a high mortality rate,which is an important factor affecting the prognosis of CTD patients.In recent years,significant progress has been made in the research on the pathogenesis,clinical manifestations,and treatments of CTD-ITP.The use of new biological agents and platelet promoting drugs has benefited patients more.Early diagnosis,grading of bleeding severity and risk assessment,as well as personalized and standardized treatment are key to achieve a good prognosis.