Therapeutic Dilemmas in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Diffuse Alveolar Hemorrhage and Venous Thromboembolism.

objectiveDiffuse alveolar hemorrhage (DAH) is a life-threatening presentation of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Patients with AAV are at an increased risk of venous thromboembolic events (VTEs). These manifestations can co-occur; however, the prognosis and management of these patients are poorly understood.

methodsIn this retrospective observational study, we included patients diagnosed with AAV who presented with both DAH and VTE during the same outpatient visit or in the same inpatient treatment episode.

resultsAmong 121 patients with proven DAH secondary to AAV managed at our institution, 14 (11.6%) were also diagnosed with VTE. Patients were predominantly men (71%) with median age at diagnosis of 67 (interquartile range 56-72) years. Five patients were diagnosed with DAH followed by VTE, four patients were diagnosed with VTE before developing DAH (T-AAV), and five patients were diagnosed with VTE and DAH simultaneously (HT-AAV). Inferior vena cava filters were placed in 13 patients (93%). Although most patients received systemic anticoagulation within 30 days of DAH diagnosis, anticoagulation management was individualized based on clinical presentation. One patient with preexisting interstitial lung disease died of respiratory failure after initially presenting with pulmonary embolism and later developing DAH during hospitalization. Bleeding complications, including recurrent DAH or retroperitoneal hematoma, were observed in six patients (43%). Three patients (21%) experienced progression or recurrence of VTE within 90 days.

conclusionManagement of patients with DAH and VTE is challenging and should be guided by the severity of DAH, additional organ manifestations of AAV, and risk stratification of the presenting VTE.