Rituximab—A Drug with Many Facets and Cures: A Treatment for Acute Refractory Hypoxemic Respiratory Failure Secondary to Severe Granulomatosis with Polyangiitis

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.

Granulomatosis with polyangiitis preceded with pyoderma gangrenosum in 41-year-old man

“…4 The mainstay of DAH treatment is the rapid administration of immunosuppressive agents �cy� clophosphamide, prednisone, and ritu�imab� and in severe cases plasmapheresis. 3,4,16,17 This treatment leads to remis� sion of the disease in 90% of cases. 18 There have been reports of the risks of developing interstitial pulmonary fibrosis fol� lowing a history of bleeding into the alveoli of patients with the AAV.…”

“…The most common skin lesions are palpable purpura, necrotic ulcera� tions, papules and nodules with many histological patterns: leukocytoclastic vasculitis, granulomatous vasculitis, and palisading granulomas. 2,3 The prevalence of GPA in Europe is 2.1-14.4 cases per million population per year. 4 Skin lesions in the course of GPA are not characteristic symptoms, but may be present in as many as 15%-50% of cases of the disease and suggest cutaneous GPA as in the case described below.…”