A novel therapeutic strategy using extracorporeal membrane oxygenation in patients with anti-neutrophil cytoplasmic antibodies-associated vasculitis: a case report and literature review

Abstract

Diffuse alveolar hemorrhage (DAH) secondary to anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) often results in severe respiratory failure which requires emergent management. In patients who are resistant to traditional mechanical respiratory support, extracorporeal membrane oxygenation (ECMO) can be used to maintain gas exchange, thereby providing time for the administration of immunosuppressive therapy to control the inflammation. Herein, we report the application of ECMO to support an adult patient with AAV complicated by severe respiratory failure due to DAH. Similar cases in the literature were identified and discussed. The patient in our case study was successfully treated with ECMO in the acute phase and relieved by immunosuppressive therapy after withdrawal of ECMO. A search in the PubMed database revealed 32 similar cases with DAH, of which 11 cases were microscopic polyangiitis (MPA), 2 cases were eosinophilic granulomatosis with polyangiitis (EGPA), and 19 cases were granulomatosis with polyangiitis (GPA). These patients were all treated with ECMO. Therefore, to date, we identified 33 patients who were effectively treated with ECMO, including 13 (39.4%) males and 20 (60.6%) females, with a ratio of 1:1.54. The average age was 32.4±17.5 and 36.0±16.1 years for males and females, respectively (t=0.610, P=0.547). Most patients received ECMO on the first day of admission to the intensive care unit (ICU) and it appeared that early initiation of ECMO was associated with a shorter duration of ECMO. In general, complications of ECMO in these patients were mild and were not often seen in the clinical setting. This study suggested that early recognition of respiratory failure and referral for ECMO are vital to achieve a satisfactory outcome in AAV patients with DAH.

Keywords: Anti-neutrophil cytoplasmic antibodies-associated vasculitis (ANCA-AAV); case report; diffuse alveolar hemorrhage; extracorporeal membrane oxygenation (ECMO); respiratory failure.

Figure 1

Chest image of a case of AAV. (A,B) CT scan of the chest showing interstitial fibrosis in both bilateral lower lung fields. (C) Chest radiograph showing bilateral diffuse alveolar opacities. AAV, ANCA-associated vasculitis; ANCA, anti-neutrophil cytoplasmic antibodies; CT, computed tomography.

Figure 2

Bronchial mucosal hyperemia. (A) View through the bronchoscope shows intratracheal bleeding. (B) Stained bloody secretions. (C) Gross hematuria was observed.

Figure 3

Chest radiography monitoring of a patient with AAV. (A) A chest radiograph taken at the conclusion of ECMO therapy. (B) CT scan of the chest after 19 days in hospital, and (C) after 1 month. AAV, ANCA-associated vasculitis; ANCA, anti-neutrophil cytoplasmic antibodies; ECMO, extracorporeal membrane oxygenation; CT, computed tomography.