The role of extra-corporeal membrane oxygenation (ECMO) in the treatment of diffuse alveolar haemorrhage secondary to ANCA-associated vasculitis: report of two cases and review of the literature
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Diffuse alveolar haemorrhage (DAH) secondary to anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a rare life-threatening condition presenting with severe respiratory failure. The management of AAV-related DAH consists of remission induction immunosuppressive therapy, which requires time to be effective, with significant fatality rates despite appropriate treatment. Extracorporeal membrane oxygenation (ECMO) can support gas exchanges providing the time necessary for immunosuppressive treatment to control the underlying disease in cases refractory to the conventional ventilation techniques. Despite severe preexisting bleeding has been considered a relative contraindication, ECMO has proven to be life-saving in several cases of respiratory failure associated with pulmonary haemorrhage due to various causes, including AAV. We reviewed the clinical presentation and course of two patients affected by AAV-related DAH treated at our Institution between 2012 and 2017, whose management required the use of veno-venous ECMO. We reviewed the current literature on the role of ECMO in the support of these patients. In both patients, ECMO provided life support and allowed disease control, in combination with immunosuppressive treatment. Despite systemic anticoagulation, clinical improvement was achieved without exacerbation of the pulmonary bleeding. We performed a literature review, and summarized available data confirming the effectiveness and safety of ECMO in AAV-related DAH. ECMO has a life-saving role in the management of patients with severe respiratory failure due to ANCA-associated pulmonary capillaritis.
KeywordsANCA-associated vasculitides Extra-corporeal membrane oxygenation Diffuse alveolar haemorrhage Respiratory failure
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Conflict of interest
The authors declare they have no conflict of interest.
- 2.Haworth SJ, Savage CO, Carr D, Hughes JM, Rees AJ (1985) Pulmonary haemorrhage complicating Wegener’s granulomatosis and microscopic polyarteritis. BMJ (Clin Res Ed) 290(6484), 1775–1778Google Scholar
- 3.Lauque D, Cadranel J, Lazor R, Pourrat J, Ronco P, Guillevin L, Cordier JF (2000) Microscopic polyangiitis with alveolar hemorrhage. A study of 29 cases and review of the literature. Groupe d’Etudes et de Recherche sur les Maladies “Orphelines” Pulmonaires (GERM"O"P). Medicine 79(4):222–233CrossRefPubMedGoogle Scholar
- 20.Peek GJ, Mugford M, Tiruvoipati R, Wilson A, Allen E, Thalanany MM et al (2009) Efficacy and economic assessment of conventional ventilatory support versus extracorporeal membrane oxygenation for severe adult respiratory failure (CESAR): a multicentre randomised controlled trial. Lancet 374(9698):1351–1363CrossRefPubMedGoogle Scholar
- 39.Hohenforst-Schmidt W, Petermann A, Visouli A, Zarogoulidis P, Darwiche K, Kougioumtzi I et al (2013) Successful application of extracorporeal membrane oxygenation due to pulmonary hemorrhage secondary to granulomatosis with polyangiitis. Drug Des Devel Ther 7:627–633PubMedPubMedCentralGoogle Scholar
- 42.Vanoli J, Riva M, Vergnano B, D’Andrea G, L’Imperio V, Pozzi MR et al (2017) Granulomatosis with polyangiitis presenting with diffuse alveolar hemorrhage requiring extracorporeal membrane oxygenation with rapid multiorgan relapse: a case report. Medicine, 96(13), e6024Google Scholar
- 44.Reinhold-Keller E, Kekow J, Schnabel A, Schmitt WH, Heller M, Beigel A et al (1990) Influence of disease manifestation and antineutrophil cytoplasmic antibody titer on the response to pulse cyclophosphamide therapy in patients with Wegener’s granulomatosis. Arthritis Rheum 37(6):919–924CrossRefGoogle Scholar
- 45.Myers JL (1993) Diagnosis of drug reactions in the lung. Monogr Pathol 36:32–53Google Scholar
- 48.Brunet F, Belghith M, Mira JP, Lanore JJ, Vaxelaire JF, Dall’ava Santucci J et al (1993) Extracorporeal carbon dioxide removal and low-frequency positive-pressure ventilation. Improvement in arterial oxygenation with reduction of risk of pulmonary barotrauma in patients with adult respiratory distress syndrome. Chest 104(3):889–898CrossRefPubMedGoogle Scholar