Early Reduction in Therapeutic Plasma Exchange for ANCA-Associated Vasculitis

Introduction: ANCA-associated vasculitis (AAV) is an autoimmune disease characterized by necrotizing inflammation of small blood vessels and the presence of antineutrophil cytoplasmic antibodies. The standard treatment for severe AAV includes TPE. The role of therapeutic plasma exchange remains debated, in terms of optimal session number and clinical benefit. Case Description: A 44-year-old woman with no past medical history was admitted to the emergency room due to dyspnea and acute kidney injury KDIGO stage 3. Her symptoms began four months earlier with intermittent myalgias and arthralgias. She was hospitalized twice and treated for community-acquired pneumonia. A level of SCr 1.2 mg/dL was reported one month before admission. Upon arrival, the patient was anuric, with a blood pressure of 130/90 mmHg, and oxygen saturation of 83%. Laboratory showed SCr 6.0 mg/dL, BUN 91 mg/dL, K 5 mEq/L, and metabolic acidosis. Urinalysis revealed acanthocytes. A diagnosis of rapidly progressive glomerulonephritis was made, and renal replacement therapy was initiated. ANCA testing was positive, with MPO 3(+), and anti-GBM (-). She received three pulses of IV methylprednisolone, followed by initiation of cyclophosphamide and three sessions of therapeutic plasma exchange. Over the following days, renal function improved, with a serum creatinine of 2.3 mg/dL one month after discharge. Discussion: The use of therapeutic plasma exchange and cyclophosphamide in patients who require dialysis and alveolar hemorrhage typically follows a 7–10 session. In our patient, the shortened use of therapeutic plasma exchange, combined with cyclophosphamide, helped contain the damage and improve renal function. This case supports the potential benefit of a shortened TPE.Figure 2.