Rituximab—A Drug with Many Facets and Cures: A Treatment for Acute Refractory Hypoxemic Respiratory Failure Secondary to Severe Granulomatosis with Polyangiitis
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. 1. 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. It is associated with ANCA. Its current incidence is unknown; however, 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. 2. Case Presentation A 32-year-old Caucasian male with a past medical history of GPA presented with fatigue, dry cough, myalgia, and arthralgia since 2 weeks. Three days prior to presentation, he developed progressive shortness of breath. The patient did not have any hemoptysis on presentation. Previous medications included cyclophosphamide and prednisone. However, cyclophosphamide was held about a week before admission when the patient was noted to have significant hematuria, proteinuria, and possible hemorrhagic cystitis. The patient was continued on prednisone. The patient at admission was initially placed on noninvasive ventilation Bipap for acute hypoxemic respiratory failure. Vital signs at admission were as following: temperature 99.2F, heart rate 120–130 beats/min normal sinus rhythm, blood pressure 109/70？mmHg, and respiratory rate 28–34/min. Oxygen saturation was 90% on bipap (IPAP-14, EPAP-6). Chest X-ray showed bilateral diffuse patchy infiltrates (see
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