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

Abstract

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.

Antineutrophil cytoplasmic antibody-associated vasculitis with alveolar hemorrhage and ruptured renal aneurysm

“…Although the lung is the most commonly affected organ in GPA, alveolar hemorrhage and ARF are rare clinical manifestations that carry an extremely high mortality rate, with an incidence of only 5% to 10% in these patients. [7][8][9][10][11] High-risk factors, including infections, environment, or genetic predisposition, may trigger an inflammatory response that involves the release of ANCA. [12] ANCA-activated neutrophils secrete proinflammatory cytokines and release reactive oxygen species to amplify vasculitis and disrupt the vascular wall, migrate into lung compartments through the alveolar-capillary membrane, and finally reach alveolar spaces mediated by CXC chemokines.…”

“…Although the lung is the most commonly affected organ in GPA, alveolar hemorrhage and ARF are rare clinical manifestations that carry an extremely high mortality rate, with an incidence of only 5% to 10% in these patients [7–11] . High-risk factors, including infections, environment, or genetic predisposition, may trigger an inflammatory response that involves the release of ANCA [12] .…”

Antineutrophil Cytoplasmic Antibody-associated Vasculitis With Alveolar Hemorrhage and Ruptured Renal Aneurysm: a Case Report and Literature Review

“…Although the lung is the most commonly affected organ in GPA, alveolar hemorrhage and ARF is a rarely clinical manifestation that carries an extremely high mortality with incidence of only 5-10% in these patients [7][8][9][10][11]. High risk factors including infections, environment or genetic predisposition may trigger an in ammatory response that involves the release of ANCA [12].…”

ANCA-assoziierte Vaskulitiden: Diagnose und Therapie von Notfallsituationen

“…Ergebnisse einer kleinen Fallserie legen einen Nutzen der Plasmapherese auch bei einer DAH nahe [27] , jedoch verhindert auch dieses additive Therapieverfahren letale Verl ä ufe leider nicht [44] . Kasuistisch wurde zudem von einem positiven Eff ekt einer Applikation von Surfactant oder Faktor VII berichtet [10,20] . Bei schwerer respiratorischer Insuffi zienz wird in vielen F ä llen eine Intubation und maschinelle Beatmung erforderlich.…”