Recurrent pulmonary embolism in antiphospholipid syndrome

A 22-year old women presented with exertional dyspnea, chest pain, fever and hemoptysis. Chest radiography revealed bilateral, peripheral lung consolidations with left sided pleural effusion (Panel A). Echocardiography showed right ventricular dysfunction. Five weeks earlier the patient had been diagnosed by CT-scan with central pulmonary embolism (PE) and deep-vein thrombosis followed by treatment with the direct oral anticoagulant (DOAC) rivaroxaban 15 mg twice daily.

Segmental defects in lung perfusion scan (Panel B, anterior scan) revealed a high probability of recurrent bilateral PE. After initial low molecular heparin and i.v. antibiotic therapy treatment was switched to the vitamin K antagonist (VKA) phenprocoumon without relapse. Persistent antiphospholipid antibodies yielded the diagnosis of antiphospholipid syndrome (APS).

Several current case-series focused on the question whether DOACs in patients with APS are a safe and non-inferior alternative to VKAs. Two phase III, prospective, randomized controlled non-inferiority open-label clinical trials are dealing with this question. TRAPS (rivaroxaban in thrombotic antiphospholipid syndrome) is still recruiting. Results of RAPS (rivaroxaban versus warfarin in patients with thrombotic antiphospholipid syndrome) are supposed to be presented in 2016.