Management of pulmonary hypertension in secondary antiphospholipid syndrome

Systemic lupus erythematosus (SLE) is the prototypic multisystem autoimmune disorder with  a broad spectrum of clinical presentations encompassing almost all organs and tissues. The extreme heterogeneity of the disease has led some investigators to propose that SLE represents a syndrome rather than a single disease. Antiphospholipidsyndrome (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). APS may be associated with other diseases, mainly systemic lupus erythematosus (SLE). Different groups of pulmonary hypertension (PH) can complicate SLE due to various etiologies. One of the well-known established pathogenesis is chronic thromboembolic pulmonary  hypertension (CTEPH). 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. The majority of patients experience substantial relief from symptoms and near normalization of hemodynamics. Patients who do not undergo PEA or suffer from persistent or recurrent PH after PEA (post-PEA PH) face a poor prognosis.