The significance of plasma exchange in the treatment of double-positive for ANCA and anti-GBM disease

Introduction: Double-positive anti-glomerular basement membrane (anti-GBM) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is very rare with high mortality. We present a case of steroid-refractory double-positive vasculitis who was successfully treated with plasma exchange (PE) and cyclophosphamide (CPA). Case: A heretofore healthy 78-year-old woman who had a month history of hemoptysis and dyspnea visited to a primary care. She was diagnosed with alveolar hemorrhage and rapidly progressive glomerulonephritis, and received steroid pulse therapy. However, she progressed to severe respiratory failure, and she was transferred to our hospital. Laboratory test revealed double-positivity of myeloperoxidase-ANCA and anti-GBM. Treatment with PE and CPA administration were underwent, which resulted in the improvement of her systemic conditions (fig). Discussion: Double-positive vasculitis is a rare disease, progresses rapidly, exhibits high mortality of 40%, and frequently relapses. Based on our own literature search, the double-positive cases have been published in 45 articles. The survival rate was 74% in double-positive cases who were treated with both immunosuppressant and PE, whereas the counterpart was 44% who were treated with immunosuppressant only. In conclusion, our report suggested that early induction of PE may be a principal strategy for the treatment of this fulminant, lethal disease.