Abstract: TH-PO439

Eculizumab and ECMO Rescue-Therapy of Most Severe ARDS in a Young Boy with Goodpasture’s Syndrome

Session Information

Category: Nutrition, Inflammation, and Metabolism

  • 1401 Nutrition, Inflammation, Metabolism

Author

  • Gross, Oliver, University Medicine Goettingen, Goettingen, Germany
Background

Goodpasture’s syndrome is a life-threatening autoimmune disease characterized by the presence anti–glomerular basement membrane (GBM) antibodies, rapid progressive glomerulonephritis and/or pulmonary hemorrhage.

Methods

In a 17-year old boy, hydrocarbon exposure and additional severe bacterial infection triggered pulmonary hemorrhage due to Goodpasture’s syndrome resulting in acute respiratory distress syndrome (ARDS). Within one day after hospital admission, the patient required extracorporeal membrane oxygenation (ECMO).

Results

Despite steroid-pulse and plasmapheresis, ARDS further deteriorated. Eleven days after admission, the boy was in a pre-final stage. At last, we decided to block the complement-driven lung damage by a single dose of Eculizumab. Three days after, lung-failure has stabilized in a way allowing us to initiate Cyclophosphamide-therapy. As mechanical ventilation further triggers Goodpasture-epitope exposure, the boy was taken from pressure support - breathing spontaneously by the help of maximal ECMO therapy. After a total of 24 days, ECMO could be stopped and pulmonary function further recovered within three weeks.

Conclusion

Our findings suggest that [1] Eculizumab can halt complement-driven organ-damage in life-threatening Goodpasture’s syndrome and [2] lung-protective early withdrawal from pressure support by the help of ECMO can prevent further harm to the lung-tissue. Both therapeutic options take Goodpasture’s pathogenesis into account and might serve as an important tool in otherwise hopeless situations to prevent further organ-damage and to gain time until the established immunosuppressive therapy works in otherwise fatal autoimmune-diseases.