Granulomatosis with Polyangiitis (GPA) is a rare systemic anti neutrophil cytoplasmic antibody (ANCA-) associated granulomatous vasculitis of the small and medium sized blood vessels. Diffuse alveolar hemorrhage (DAH) is a rare life-threatening complication of GPA. In our patient, cyclophosphamide was held secondary to complications of acute kidney injury, hematuria, and concern for a possible hemorrhagic cystitis. However, during the workup for hematuria the patient acutely developed respiratory failure and was found to have DAH. The patient was initially supported with mechanical ventilation volume control mode, steroids, and plasma exchange. With no improvement of oxygenation, the mode of ventilation was changed to airway pressure release ventilation (APRV) and the patient was started on rituximab. The patient clinically improved over the next few days, was able to be extubated, and was transferred out of the intensive care unit.

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