Abstract

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.

Highlights

  • Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis that affects small and medium sized blood vessels with granuloma formation

  • We report a case of GPA with refractory hypoxemia secondary to diffuse alveolar hemorrhage that was treated with rituximab and supportive treatment

  • In a recent effort to have vasculitis nomenclature free of eponyms it was changed to the current name of GPA [2]

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Summary

Introduction

Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis that affects small and medium sized blood vessels with granuloma formation. It typically produces granulomatous inflammation of the upper, lower respiratory tracts and necrotizing glomerulonephritis in the kidneys. Its current incidence is unknown; its prevalence is believed to be 3/100,000 people in the USA. Diffuse alveolar hemorrhage (DAH) is identified in 25% of the patients with severe GPA [1]. We report a case of GPA with refractory hypoxemia secondary to diffuse alveolar hemorrhage that was treated with rituximab and supportive treatment

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