Antiphospholipid Syndrome and the Lungs

Abstract

Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterised by recurrent thromboembolic events (arterial or venous) and/or morbidity in pregnancy (fetal loss, premature birth, or recurrent embryonic losses) in the presence of laboratory evidence of antiphospholipid antibodies (aPL). APS is a multisystem disorder. Several lung manifestations may develop in patients with APS including pulmonary embolism (PE)/infarction; thromboembolic and non-thromboembolic pulmonary hypertension (PH) (pulmonary arterial hypertension); pulmonary microthrombosis; acute respiratory distress syndrome associated with catastrophic APS; diffuse alveolar haemorrhage; and pulmonary capillaritis. Postpartum syndrome and cryptogenic fibrosing alveolitis (CFA) can be associated with APS. Pulmonary manifestations are relatively rare but are more likely to be life-threatening compared with other complications of APS. Particularly in the presence of aPL, pulmonary manifestations should be suspected in any systemic lupus erythematosus patient with clinical findings such as chest pain, dyspnoea, tachypnoea, and haemoptysis. Early diagnosis and treatment of pulmonary manifestations in APS are essential for improving mortality rates in patients with this condition. The purpose of this review is to assess current evidence around the diagnosis, prognosis, and management of patients with common and rare pulmonary manifestations of APS.