Primary antiphospholipid syndrome associated Liebman-Sachs endocarditis leading to diffuse alveolar hemorrhage: A case report

Abstract

A 26 year old female presented for recurrent blood tinged sputum during the previous year with development of frank hemoptysis three days prior to admission. Diffuse alveolar hemorrhage (DAH) was confirmed with serial lavages. The patient had no history of autoimmune disease, vascular thrombosis or pregnancy morbidity including miscarriages or pre-eclampsia. High dose steroids were initiated along with noninvasive ventilatory support. Transthoracic echocardiogram showed severe mitral regurgitation and a vegetation on the mitral valve; transesophageal echocardiogram determined the lesion highly suggestive of Libman-Sachs endocarditis. Blood cultures were negative. Immunological evaluation established the patient was negative for: anti-nuclear antibody, anti-double-stranded DNA antibody, rheumatoid factor, anti-smith antibody, anti-cyclic citrullinated peptide, anti-neutrophil cytoplasmic antibodies, anti-glomerular basement membrane antibodies. Further evaluation revealed elevated levels of anticardiolipin immunoglobulin G and anti-beta 2 glycoprotein immunoglobulin G which continued to increase for months after hospitalization. She was diagnosed with DAH secondary to acute mitral regurgitation caused by Libman-Sachs endocarditis in the presence of primary antiphospholipid antibody syndrome.DAH is an important disease to understand given its high mortality rate. Few case reports relating the presence of Libman-Sachs endocarditis induced by antiphospholipid antibody syndrome leading to DAH have been published. Unique here is the absence of rheumatologic markers thus supporting a diagnosis of primary antiphospholipid antibody syndrome (APS). This patient had no findings associated with rheumatological disorders potentially making this diagnosis easily overlooked. This case further illustrates the importance of evaluating patients with APS presenting with DAH as there are multiple etiologies that lead to this pathology thus different treatment avenues are to be considered during management.

A Fatal Case of Diffuse Alveolar Hemorrhage in the Setting of Systemic Lupus Erythematosus: A Case Report and Review of Noninfectious Causes of Acute Pulmonary Hemorrhage in Adults

“…Chronic and acute mitral valve disease, though less commonly considered, has long been recognized as the sole cause of alveolar hemorrhage [ 18 , 19 ]. However, some of the reports of mitral valve regurgitation associated with DAH have occurred in patients with other conditions associated with SLE, such as Libman–Sacks endocarditis and APAS [ 20 , 21 ].…”

Life-threatening hemoptysis accompanied by internal thoracic artery aneurysms in a 28-year-old perinatal woman: a case report

“…A prospective study with 606 patients was performed to investigate the etiology of hemoptysis and revealed that compared with CT scans (77.3% of cases) and bronchoscopy (48.7% of cases), the combination of CT scan and bronchoscopy was able to diagnose the etiology of the hemorrhage in 83.9% of cases [2]. However, there are still some rare causes that can result in a misleading clinical diagnosis, such as antiphospholipid antibody syndrome (APS) [3] and pseudoaneurysm [4]. Additionally, in some cases, the diagnostic procedures involving bronchoscopy were difficult and risky.…”

Human umbilical cord-derived mesenchymal stem cells and nitric oxide modulators attenuate the proinflammatory cytokine pattern in experimental antiphospholipid syndrome in mice

“…To facilitate the diagnosis of aPL, a classification that combines clinical signs and laboratory criteria is used. Three types of aPL have been established: lupus anticoagulant (LA), anticardiolipin antibodies (aCL) and antibodies to b2 glycoprotein I (ab2GPI) [1][2][3][4]. APS develops as a separate clinical syndrome (primary APS) or as a manifestation of another disease (secondary APS), mainly in systemic lupus erythematosus (SLE) or other diseases with affection of vascular endothelium [2,3].…”