Background/Purpose:

Diffuse alveolar haemorrhage (DAH) is a rare and potentially fatal complication of the systemic vasculitides and may present directly to the intensivist as a severe acute respiratory distress syndrome (ARDS) with reported mortality of 12- 60%. Whilst a severe respiratory failure (SRF) therapy strategy incorporating extracorporeal membrane oxygenation (ECMO) improves outcomes in ARDS, use of ECMO in DAH is often considered to be relatively contraindicated due to the requirement for systemic anticoagulation.

Methods:

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

Results:

The case series is described in table 1. Median lung injury score (LIS) was 3.5. Three patients required ECMO (median duration 8 days) and all received immunosuppression. One patient received normal heparin protocol to target APTTr 1.5-2 whilst two patients had 48 hours of ECMO with no heparin followed by sub-therapeutic low dose heparin. ICU survival was 100% and six months survival was also 100%. There were no exacerbations of pulmonary haemorrhage, no new events of extra-pulmonary haemorrhage and no clotting complications.

Table 1. MV, mechanical ventilation; LOS, length of stay; GPA, Granulomatosis and Polyangiitis (Wegener’s Granulomatosis); MPA, Microscopic Polyangiitis; PEx, Plasma exchange; MEP, methylprednisolone; HD, Continuous veno-venous haemodialysis; CYC, Cyclophosphamide

The ELSO database contains 78 patients (adult, 59; paediatric, 19) with pulmonary vasculitides who received ECMO. 43 had a diagnosis of Granulomatosis and Polyangiitis (GPA), whereas the remaining diagnoses included hypersensitivity angiitis, Goodpasture’s syndrome and thrombotic microangiopathy. The median age was 23 years (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. 

Conclusion:

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

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/extra-corporeal-membrane-oxygenation-and-diffuse-alveolar-haemorrhage-a-single-centre-case-series-and-analysis-of-the-elso-database/