Haematologica. 1993 Sep-Oct;78(5):313-8.
Since 1989 we have been promoting an Italian Registry of patients with APA (IR-APA), and we report here the analysis on 319 cases (80 males and 239 females, aged from 2 to 76 years, median 31 years) from 22 institutions.
A two-page questionnaire was prepared, divided into 4 parts: 1. personal data, 2. associated conditions, 3. biology (for the diagnosis of APA), 4. history of thromboembolism, haemorrhage and pregnancy. Every information was entered into a computerized registry.
The IR-APA included 207 cases of idiopathic APA and 112 cases of APA secondary to SLE. Seventy-one out 207 (34.3%) and 47 out of 112 (42%) cases experienced arterial and/or venous thrombosis, respectively (not significant). Among patients with a thrombotic history, venous thrombosis were experienced by 60.5% vs 57.4%, arterial thrombosis by 26.7% vs 31.9%. Venous thrombosis of the legs and arterial thrombosis of the cerebral vessels were the most common types of thrombosis in both groups. Thrombocytopenia was present in 27.7% of idiopathic APA vs 22.4% of secondary APA (not significant). Four patients experienced major haemorrhagic events.
This retrospective study shows that the prevalence of thrombosis and thrombocytopenia in patients with idiopathic APA is similar to that observed in patients with APA secondary to SLE.
自1989年以来，我们一直在推动意大利原发性醛固酮增多症（APA）患者登记处（IR-APA）的工作，在此我们报告对来自22个机构的319例患者（80例男性和239例女性，年龄从2岁至76岁，中位数为31岁）的分析。
准备了一份两页的问卷，分为4部分：1.个人数据，2.相关病症，3.生物学指标（用于APA的诊断），4.血栓栓塞、出血和妊娠史。所有信息都录入了计算机化登记处。
IR-APA包括207例特发性APA和112例继发于系统性红斑狼疮（SLE）的APA。207例中的71例（34.3%）和112例中的47例（42%）分别经历了动脉和/或静脉血栓形成（无显著差异）。在有血栓形成病史的患者中，静脉血栓形成的发生率为60.5%对57.4%，动脉血栓形成为26.7%对31.9%。两组中，腿部静脉血栓形成和脑血管动脉血栓形成是最常见的血栓形成类型。特发性APA患者中血小板减少症的发生率为27.7%，继发于SLE的APA患者中为22.4%（无显著差异）。4例患者发生了严重出血事件。
这项回顾性研究表明，特发性APA患者中血栓形成和血小板减少症的患病率与继发于SLE的APA患者中观察到的患病率相似。
