The clinical characteristics of antiphospholipid syndrome associated with chronic thromboembolic pulmonary hypertension

Abstract

Objective: To analyze the clinical characteristics of antiphospholipid syndrome (APS) patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods: A total of 22 APS patients with CTEPH were enrolled in our study, who were admitted in Peking Union Medical College Hospital from January 2012 to August 2018. Diagnoses were confirmed by computed tomographic pulmonary angiography (CTPA), or pulmonary angiography. Demographic characteristics, clinical manifestations, laboratory tests, therapy, World Health Organization (WHO) functional class were retrospectively collected. Results: There were 15 females and 7 males with a median age of 29-year-old. Chest pain (6 cases), dyspnea on exertion (22 cases), cough (6 cases) and hemoptysis (9 cases) were the most common clinical manifestations. Lupus anticoagulant (LA), anticardiolipin (ACL) antibodies and anti-beta 2 glycoprotein Ⅰ (anti-β(2) GPⅠ) antibodies were all positive in 12 patients, two of three antibodies positive in 5 patients, only one positive in 5 patients. The WHO functional classes were Ⅱ-Ⅳ before treatment. Anticoagulants were administrated in all patients. After multidisciplinary evaluation, 9 patients underwent pulmonary thromboendarterectomy (PTE), who all had a good outcome. Symptoms in eleven over thirteen patients with only anticoagulants improved. Three patients developed cardiac deterioration while other 3 patients died of right heart failure during follow-up. Conclusion: Pulmonary embolism is one of the most common thrombotic events in APS patients. It is important to recognize symptoms and signs related to pulmonary embolism and start anticoagulation as soon as possible. Standard anticoagulation improves symptoms but can't reverse the process of pulmonary hypertension. Some patients may benefit from PTE after anticoagulation and multidisciplinary evaluation.