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SP0006 A Difficult to Treat Case with Relapsing Granulomatosis with Polyangiitis
  1. R. Cartin-Ceba
  1. Mayo Clinic, Rochester, United States


A 34 year-old woman with known history of granulomatosis with polyangiitis (GPA) is evaluated due to new onset dyspnea, hemoptysis, malaise, fever and migratory arthralgias. Patient had been diagnosed with GPA approximately two years before current presentation when she was evaluated due to recurrent sinusitis, indeterminate pulmonary nodules and acute renal failure in the setting of an active urinary sediment and a positive c-ANCA/PR3. A kidney biopsy at that time showed necrotizing pauci-immune glomerulonephritis. She was initially treated 4 months with oral cyclophosphamide and tapering oral corticosteroids for induction of remission and then switched to azathioprine for maintenance of remission which she had been taking for approximately 18 months prior to current presentation. Patient was hospitalized given her new symptoms and also due to the presence of new onset hypoxemia requiring oxygen supplementation. Given her new onset dyspnea and hemoptysis, the patient underwent chest imaging that revealed the presence of bilateral alveolar opacities. Her hemoglobin was slightly reduced from her baseline, inflammatory markers were significantly elevated and her creatinine had doubled from baseline with evidence of active urinary sediment. A diagnostic bronchoscopy was performed from the right middle lobe and showed progressively bloodier return after repeated aliquot injections. BAL studies did not reveal the presence on an infectious process. Her condition deteriorated requiring intubation and mechanical ventilation; in addition, her renal function also worsened requiring initiation of renal replacement therapy. Patient's spouse raised concerns with repeated cyclophosphamide therapy and fertility and the patient was then treated with high doses of corticosteroids and 4 infusions of rituximab. Patient was also treated with 6 sessions of plasma exchange. Patient's respiratory failure resolved and her renal function improved and was able to come off hemodialysis therapy before discharge from the hospital. Patient achieved complete remission 4 months after discharge.

Disclosure of Interest None declared

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