2003
DOI: 10.1007/bf03040505
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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 bo… Show more

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Cited by 8 publications
(5 citation statements)
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“…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.…”
Section: Literature Review and Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…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.…”
Section: Literature Review and Discussionmentioning
confidence: 99%
“…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] .…”
Section: Literature Review and Discussionmentioning
confidence: 99%
“…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].…”
Section: Disscussion and Conclusionmentioning
confidence: 99%
“…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.…”
Section: Diff Use Alveol ä Re H ä Morrhagie (Dah) and Inzidenz Und Progunclassified