Thromboendarterectomy in a patient with Systemic Lupus and antiphospholipid Syndrome, lessons learned from a complex disease interaction

Systemic lupus erythematosus (SLE) is the prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations encompassing almost all organs and tissues(1). Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis and pregnancy morbidity in the presence of pathogenic autoantibodies known as antiphospholipid antibodies (aPL)(2). Chronic thromboembolism is one of the well-known established pathogenesis of pulmonary hypertension, known as chronic thromboembolic pulmonary hypertension (CTEPH)(3). APS may be also associated with other diseases, mainly systemic lupus erythematosus (SLE). The presence of secondary APS in SLE patients further aggravate the condition due to recurrent venous thromboembolic showers to the pulmonary vasculature. Pulmonary endarterectomy (PEA) is the treatment of choice for CTEPH with lifelong anticoagulation(4). We herein report a rare cause of CTEPH in a 42-year-old Egyptian man who presented with dyspnea WHO-FC III. The patient was diagnosed as a case of CTEPH due to secondary APS. He underwent PEA and was discharged on lifelong anticoagulation. Clinical follow-ups thereafter showed improvement of functional capacity and pulmonary artery pressures. In conclusion, management of such cases was combination of standard treatment of CTEPH, in addition to specific management of secondary APS to avoid recurrence of the disease.