Long-Term Outcome of Immunosuppression in a Patient With Immunoglobulin A Nephropathy and Serum-Positive Antineutrophil Cytoplasmic Antibodies

Abstract

The concurrent occurrence of immunoglobulin A nephropathy (IgAN) and serum-positive antineutrophil cytoplasmic antibodies (ANCA) is a rare clinical entity frequently associated with more severe disease progression. Without appropriate intervention, affected individuals may rapidly advance to end-stage kidney disease (ESKD), necessitating renal replacement therapy. Early initiation of intensive induction immunosuppressive therapy has the potential to enhance renal outcomes, which can be maintained over time with suitable maintenance regimens. In this report, we describe the management of a patient with pre-existing chronic kidney disease (CKD) and impaired baseline renal function who was treated with cyclophosphamide for induction and azathioprine for maintenance for crescentic IgAN and serum-positive ANCA. This approach resulted in substantial improvement in renal function, which was preserved over a 55-month follow-up period.