Diffuse alveolar haemorrhage and Libman-Sacks endocarditis: a rare presentation of antiphospholipid syndrome

Abstract

A 26-year-old woman with a history of idiopathic thrombocytopaenic purpura and a 1-year history of blood-streaked sputum presented after a severe episode of haemoptysis with dyspnoea. Chest imaging revealed diffuse ground glass and bronchovascular nodules. Bronchoscopy revealed bilateral diffuse alveolar haemorrhage (DAH). Sputum and bronchoalveolar lavage studies were negative for infectious aetiologies. A transthoracic echocardiogram revealed Libman-Sacks endocarditis with severe mitral regurgitation and physical examination revealed retinal artery occlusion and Osler's nodes. The patient had an increased anticardiolipin Immunoglobulin IgG and anti-B2 glycoprotein IgG, suggesting antiphospholipid syndrome (APLS). The patient was then started on high-dose methylprednisolone and had an improvement in her dyspnoea and haemoptysis. She was also started on anticoagulation as treatment for Libman-Sacks endocarditis. APLS should be considered as a possible underlying aetiology for unusual presentations of DAH with concurrent Libman-Sacks endocarditis in non-intravenous drug users with existing autoimmune disorders.

Clinical Insights into Diffuse Alveolar Hemorrhage in Antiphospholipid Syndrome

“…Yet, two other reviews of 18 and 13 patients noted a median DAH onset of 5.9 and 5.8 years, respectively, after the diagnosis of APS [25,46]. Furthermore, many patients have a delay in diagnosis [53,54], e.g., a 26-year-old female visited her primary care doctor and emergency room for multiple episodes of hemoptysis throughout 1 year before the eventual diagnosis of APS [49].…”