系统性硬化症血小板减少患者临床特点分析

Clinical Characteristics of Thrombocytopenia in Patients with Systemic Sclerosis

摘要: 目的：探讨系统性硬化症(systemic sclerosis, SSc)血小板减少患者临床特点，进一步提高对血液系统受累SSc认识。方法：分析2005年2月至2020年2月就诊于青岛大学附属医院初治274例SSc患者临床资料，据血小板是否减少分为血小板减少组和血小板正常组，比较两组患者一般情况、临床特点和实验室指标。结果：274例SSc患者，血小板减少者23例，其发病率为8.4%。血小板减少组患者发病年龄、补体C3、C4均低于血小板正常组，差异具有统计学意义(P < 0.05)；血小板减少组患者血沉升高比例、肺动脉高压发生率高于未减少组，差异有统计学意义(P < 0.05)。血小板减少组患者抗SSA抗体、抗SSB抗体阳性率高于未减少组，差异有统计学意义(P < 0.05)。结论：血小板减少组SSc患者更易合并肺动脉高压，可能与血小板功能异常引起自身免疫功能紊乱并产生自身抗体引起血管损伤及血管重塑所致。对于初治SSc患者出现补体C3、C4低，血沉快及抗SSA抗体、抗SSB抗体阳性时应警惕血小板减少的发生。

Abstract: Objective: To investigate the clinical characteristics of patients with systemic sclerosis (SSc) with thrombocytopenia, and to improve the understanding of the disease. Methods: Analysis of clinical data of 274 SSc patients in the Affiliated Hospital of Qingdao University from February 2005 to February 2020, and were divided into thrombocytopenia group and non-thrombocytopenia group according to clinical manifestations and imaging findings. Results: Of the 274 SSc patients, 23 were associated with thrombocytopenia, the incidence was 8.4%. The incidence proportion of increased erythrocyte sedimentation rate and pulmonary hypertension in the thrombocytopenia group were higher than those in the normal group, and the differences were statistically significant (P < 0.05). The positive rates of anti-SSA antibody and anti-SSB antibody in the thrombocytopenia group were higher than those in the normal group, and the difference was statistically significant (P < 0.05). Conclusions: SSc patients in the thrombocytopenia group are more likely to complicate with the pulmonary hypertension, as the qualitative platelet defects can produce the immune dysfunction and the autoantibodies causing the vascular injury and remodeling. Therefore, it’s vital to maintain great vigilance on the occurrence of thrombocytopenia when the primary SSc patients suffers the symptoms including low complement C3 and C4, rapid erythrocyte sedimentation rate and positive anti-SSA antibody and anti-SSB antibody.