Kidney Week

Abstract: FR-PO0797

Relapse During Rituximab Induction for PR3-ANCA-Associated Vasculitis with Pauci-Immune Crescentic Glomerulonephritis and Diffuse Alveolar Hemorrhage (DAH)

Session Information

• Glomerular Diseases: ANCA Vasculitis, Anti-GBM Disease, and Crescentic GN
  October 23, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Rich, Trent E., Southern Illinois University School of Medicine, Springfield, Illinois, United States

Trent E. Rich, MD

• Carey, Austin L., Southern Illinois University School of Medicine, Springfield, Illinois, United States

Austin L. Carey, MD

• Sweis, Jaleel Jerry, Southern Illinois University School of Medicine, Springfield, Illinois, United States

Jaleel Jerry Sweis, MD

• Chen, Xueguang (Gary), Southern Illinois University School of Medicine, Springfield, Illinois, United States

Xueguang (Gary) Chen, MD, PhD

Introduction

Granulomatosis with Polyangiitis (GPA) is a rare vasculitis associated with anti-PR3 antibody, which causes neutrophil activation from serine protease activation in granulocytes causing damage to small vessel endothelial cells. Manifestations include glomerulonephritis (80% of cases by two years), respiratory tract, cutaneous and musculoskeletal involvement¹. In this case, a patient with newly diagnosed GPA with GN and DAH started on Rituximab induction treatment presented with disease flare initially concerning for resistant disease.

Case Description

81-year-old male admitted with weight loss and poor oral intake with initial workup showing Hgb 9.2g/dL, creatinine of 2.2 mg/dL up from 1.2 mg/dL six months prior, microscopic hematuria and proteinuria, and lung nodules at the top of a CT Abd/Pelv; CT Chest held off due to contrast. Serologic workup significant for positive ANCA (1:640) in a c-ANCA pattern with positive PR3 antibodies.

Before renal biopsy, patient developed acute hypoxic respiratory failure, confirmed to be DAH on bronchoscopy, which responded to pulse steroids and two plasmapheresis sessions. Renal biopsy showed focally crescentic and sclerosing pauci-immune ANCA glomerulonephritis, patchy and focal interstitial and tubular fibrosis. Patient was discharged on Rituximab induction therapy with prednisone taper.

After second dose of Rituximab, patient was hospitalized for pneumonia, and continued Rituximab on discharge. After third dose, patient presented with DAH and worsened renal function which responded to steroids, plasmapheresis and cyclophosphamide before transferring to a Vasculitis Center due to concern of Rituximab failure. BAL cultures grew Pseudomonas, after treatment, he was restarted on Rituximab.

Discussion

Rituximab is first line induction therapy for GPA and relapsing vasculitis, as proven by the post-hoc analysis and further studies^2,3,4 following RAVE trial⁵ which is reflected in current guidelines⁶. The patient presented with a flare during induction therapy concerning for refractory disease, which by guidelines, Cyclophosphamide should be added, which was done. However, the relapse in disease was likely due to Pseudomonas Pneumonia. The learning point in this case is that Rituximab did not fail as induction therapy, rather a relapse due to infection. However, resistant disease is important to keep in mind during induction relapses.