Background Diffuse alveolar haemorrhage (DAH) is a rare and potentially fatal complication of the systemic vasculitides¹. DAH may present as a severe acute respiratory distress syndrome (ARDS) with reported mortality of 12- 60%^1. Whilst extracorporeal membrane oxygenation (ECMO) improves outcomes in ARDS², use of ECMO in DAH may be considered to be relatively contraindicated due to the requirement for systemic anticoagulation.

Methods A case series of 4 patients with DAH due to ANCA vasculitides diagnosed and or managed by a standardised diagnostic pathway and ARDS treatment algorithm in a single, UK SRF centre, between 2012-13. Analysis of the Extracorporeal Life Support Organisation (ELSO) database using ICD-9 codes 446.2,446.21,446.4,446.6 to report the current international experience of DAH and ECMO³.

Results 4 patients with ARDS due to systemic vasculitides were referred via the SRF pathway, but only 3 patients required ECMO (range 120 – 208 hours). 1 patient received a normal protocol heparin regime to target an APTTr of 1.5-2 whilst two patients had 48 hours of ECMO with no heparin followed by low-dose heparin. ICU survival and six months survival were 100% and there were no exacerbations of pulmonary haemorrhage and no clotting complications.

The ELSO database contains 78 patients (adult,59; paediatric,19) with pulmonary vasculitides who received ECMO. 43 had GPA, whereas the remaining diagnoses included hypersensitivity angiitis, Goodpasture's syndrome and thrombotic microangiopathy. The median age was 23 years old (IQR: 16-47). The median duration of ECMO was 190 hours (IQR: 146-282) and ICU survival was 82%. Twelve patients (15%) were reported to have thrombotic ECMO circuit complications in the context of likely conservative heparinisation.

Conclusions In this case series, ECMO offers an excellent survival rate and clinical outcomes in SRF due to ANCA-associated DAH. ELSO data supports our case series and suggests that DAH related to vasculitides should not be considered a contraindication. ECMO should be considered as supportive therapy in DAH patients with SRF not responsive to conventional therapy.

References

1. Sugimoto T et al. Pulmonary-renal syndrome, diffuse pulmonary haemorrhage and glomerulonephritis, associated with Wegener's granulomatosis effectively treated with early plasma exchange therapy. Intern Med 2007;46:49-53.

2. Efficacy and economic assessment of conventional ventilatory support versus extracorporeal membrane oxygenation for severe adult respiratory failure (CESAR): a multicentre randomised controlled trial. Lancet 2009;374:1351-63.

3. ECMO Registry of the Extracorporeal Life Support Organization (ELSO), Ann Arbor, Michigan,June (2013).

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.4191