Acute hemorrhagic respiratory failure caused by Wegener's granulomatosis successfully treated by bronchoalveolar lavage with diluted surfactant.

Wegener's granulomatosis (WG) is an idiopathic inflammatory systemic disease that can occasionally cause an acute respiratory distress syndrome. We report on a 17-year-old girl with Wegener's granulomatosis and acute hemorrhagic respiratory failure successfully treated using bronchoalveolar lavage with diluted porcine surfactant (Curosurf; 4.8 mg/mL) followed by a low-dose bolus of surfactant. The cumulative dose of surfactant was 40 mg/kg BW. The lavage with diluted surfactant and the administration of the bolus were performed with a flexible bronchoscope. The patient was ventilated during the whole procedure, stayed hemodynamically stable and showed only a very short phase of desaturation. The PaO2/FiO2 ratio increased from 54.8 to 62.4 after one hour, to 106 after 17 hours and finally to 280 after four days. The patient was extubated five days after lavage treatment, and almost normal lung function was restored after eight weeks. Bronchoalveolar lavage with diluted surfactant by flexible bronchoscopy allows selective and direct drug administration and removes airway and alveolar debris. The technique reduces the amount of surfactant needed to overcome inhibition and thereby reduces therapy costs. We conclude that this early therapeutic intervention with surfactant might help to avoid an invasive rescue therapy such as extra corporeal membrane oxygenation, thus improving outcome in terms of faster recovery of lung function.