Primary Antiphospholipid Syndrome Associated with Diffuse Alveolar Hemorrhage and Pulmonary Thromboembolism

Abstract

Antiphospholipid syndrome (APS) is clinically characterized by arterial or venous thrombosis; however, non-thromboembolic lung manifestations, such as diffuse alveolar hemorrhage (DAH), have also been previously reported. DAH is relatively common in APS patients with systemic lupus erythematosus, although it is rare in primary APS. We encountered a 78-year-old man who presented with hemoptysis and dyspnea. Chest CT showed diffuse ground-glass opacity with pulmonary thromboembolism. He was successfully treated with corticosteroids and heparin; however, DAH recurred after the corticosteroid treatment was stopped. The treatment was intricate due to the concurrent bleeding and thrombotic manifestations.

Continous Rituximab treatment for recurrent diffuse alveolar hemorrhage in a patient with systemic lupus erythematosus and antiphosholipid syndrome

“…DAH is a well known but rare complication to SLE although a more common complication to SLE than to APS. However, a few cases of APS patients with DAH have been reported [10] , [11] , [12] , [13] , [14] . Only a single case report has previously described RTX as treatment of DAH in a patient with APS without SLE [12] .…”

Clinical Insights into Diffuse Alveolar Hemorrhage in Antiphospholipid Syndrome

“…When stopped, we recommend restarting anticoagulation as soon as the acute bleeding is controlled. In cases with concurrent pulmonary embolism and hemorrhage, starting anticoagulation and corticosteroids simultaneously may be required [70]. A multidisciplinary team approach should appraise the risks and benefits, i.e., timing and dosage of safe anticoagulation [71].…”

Tromboembolismo pulmonar bilateral como presentación de un síndrome antifosfolipídico: un reporte de caso

“…Si bien se reporta que el 40% de pacientes con SAF, presentan embolia pulmonar (EP) en el transcurso de la enfermedad [9] , solo un 9% de pacientes debutan con esta condición, y la tasa es aún más infrecuente cuando se trata de una presentación bilateral [10][11][12] . Los pacientes en los que se ha encontrado trombosis bilateral de las principales arterias pulmonares, en su mayoría, suelen ser pacientes con SAF primario, que desarrollaron clínica de hipertensión pulmonar (HTP) y en quienes en el estudio angiográfico demostró afectación de las arterias pulmonares, tanto de las principales como de las lobulares, en forma de obstrucción completa o parcial [10] .…”