ANCA Vasculitis Presenting with Acute Interstitial Nephritis Without Glomerular Involvement

ANCA-associated vasculitis (AAV) with renal involvement typically causes pauci-immune glomerulonephritis. We present a case of acute interstitial nephritis (AIN) as the sole renal lesion without glomerulonephritis with myeloperoxidase (MPO) AAV. A 45-year-old female with history of Crohn's disease, gastroesophageal reflux disease (GERD) with Barrett's esophagus, pulmonary embolism, and polyarthralgias was evaluated in nephrology clinic in 2018. AIN without glomerulonephritis was first diagnosed in 2011 on renal biopsy. p-ANCA was positive with MPO titer of > 100 U/mL. Serum creatinine improved from 2.1 to 0.9 mg/dL with prednisone and azathioprine. Repeat biopsy in 2013 for worsening renal function showed AIN without glomerular involvement. Serum creatinine improved from 1.9 to 1.2 mg/dL with prednisone and cyclosporine. Crohn's disease was diagnosed in 2014. AIN was attributed to Crohn's, and cyclosporine was stopped in 2016. Adalimumab was started in 2016, without improvement in renal function or urine sediment. Attempt was made to switch proton pump inhibitor (PPI) to H2-blocker, but the latter was not tolerated. Repeat biopsy in 2/2018 showed AIN with severe fibrosis and tubular atrophy and glomerulosclerosis but no active glomerular disease. MPO titers remained high at 132 U/mL. Mycophenolic acid and prednisone were started without response, followed by rituximab for AAV-associated AIN. Serum creatinine worsened to 6.0 mg/dL in 9/2018, with plan to start peritoneal dialysis. AAV may present with isolated AIN without glomerular involvement. The rarity of this presentation may contribute to delay in appropriate management. Alternative explanations for AIN, such as Crohn's disease or PPI use should be considered with caution in the setting of high-titer ANCA positivity.