Rheumatology International

, Volume 39, Issue 2, pp 367–375 | Cite as

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

  • Paolo DelvinoEmail author
  • Sara Monti
  • Silvia Balduzzi
  • Mirko Belliato
  • Carlomaurizio Montecucco
  • Roberto Caporali
Cases with a Message


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.


ANCA-associated vasculitides Extra-corporeal membrane oxygenation Diffuse alveolar haemorrhage Respiratory failure 


Compliance with ethical standards

Conflict of interest

The authors declare they have no conflict of interest.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Rheumatology Department, IRCCS Policlinico S. Matteo FoundationUniversity of PaviaPaviaItaly
  2. 2.University of PaviaPaviaItaly
  3. 3.UOS Advanced Respiratory Intensive Care Unit, UOC Anestesia e Rianimazione 1IRCCS Policlinico S. Matteo FoundationPaviaItaly

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