Successful reintegration of a very elderly patient with rapidly progressive glomerulonephritis due to microscopic polyangiitis after multidisciplinary treatment: a case report

ANCA (anti-neutrophil cytoplasmic antibody)-associated vasculitis (AAV) is known to cause rapidly progressive renal dysfunction, as well as high mortality, especially in older patients. In Japan, a high prevalence of microscopic polyangiitis (MPA) with myeloperoxidase (MPO)-ANCA among older patients has been reported; however, standard immunosuppressive therapy does not always lead to improved outcomes in this population. Here we report the case of a 93-year-old woman who developed rapidly progressive glomerulonephritis (RPGN) due to MPA and successfully returned home after multidisciplinary treatment. She was transferred from another hospital because of antibiotic-refractory fever. Serological testing revealed MPO-ANCA positivity and a renal biopsy demonstrated crescentic glomerulonephritis. She was diagnosed with RPGN due to MPA and was initiated on standard glucocorticoid therapy, despite her advanced age. Because of severe disease activity complicated by diffuse alveolar hemorrhage, noninvasive positive pressure ventilation, rituximab, avacopan, and plasma exchange combined with hemodialysis were introduced. This multidisciplinary approach successfully induced remission of her AAV with partial recovery of renal function. After rehabilitation for mobilization, the patient was discharged home. She currently attends outpatient follow-up independently without assistance. Although very elderly patients are often excluded from clinical trials, resulting in limited evidence regarding the efficacy and safety of therapeutic agents, appropriately dose-adjusted multidisciplinary treatment based on overall clinical conditions may improve outcomes, even in this population.