16例结缔组织病合并门静脉系统血栓形成的临床分析

 目的 分析结缔组织病合并门静脉系统血栓形成病例的临床特点，提高对该病的认识。方法 回顾性分析16例结缔组织病合并门静脉系统血栓形成病例的临床特点。结果 16例患者中系统性红斑狼疮6例，原发性抗磷脂抗体综合征、类风湿关节炎、原发性干燥综合征及未定型结缔组织病各2例，混合性结缔组织病及CREST综合征各1例。男性2例，女性14例，年龄24～66岁。合并门脉高压者11例，深静脉血栓形成者4例，血栓部位以门静脉多见（9例）。急性血栓形成主要表现为急性腹痛、腹胀、便血；慢性血栓形成者以慢性腹痛、腹胀或门脉高压相关的症状为主要表现。结论 门静脉系统血栓可以为结缔组织病的首发症状或慢性并发症，应提高对该病的警惕。

Clinical analysis of 16 cases of connective tissue disease with portal venous system thrombosis

Objective To improve the understanding of the clinical characteristics of connective tissue disease with portal venous system thrombosis. Methods The clinical features of 16 patients of connective tissue disease with portal venous system thrombosis were reviewed. Results Among 16 patients, 6 cases were SLE, followed by 2 case of APS, 2 case of RA, 2 cases of pSS; 1 case of MCTD, 1 case of CREST syndrome and 2 cases of unspecific CTD. Two cases were male and 14 cases were female with the median age of 48 years. Eleven cases had portal hypertension, 4 patients had deep venous thrombosis. The most common thrombosis site was portal vein (9 cases). Acute PVST could be manifested with acute abdominal pain or distention, chronic PVST could have chronic incomplete intestinal obstruction, or hematemesis/melena related to portal hypertension. Conclusion Portal venous system thrombosis can be the initial or the complicated sign in the connective tissue disease, physicians should be alert to this disease.