Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis known to affect the respiratory and renal systems. There are a multitude of clinical manifestations, many of which are not specific to the disease, such as dysfunction of the nasal, sinus, auditory, tracheal, pulmonary, ocular, renal, cardiac, and nervous systems. As a multisystemic illness without a "classic" presentation and insidious progression, it is often a challenging diagnosis. We report and discuss a case of a 10-year-old female with no significant past medical history who presented to the emergency department with a 10-day course of worsening respiratory symptoms. As her respiratory and clinical status began to precipitously decline, the decision was made to intubate the patient, which was performed without issue. Unfortunately, attempts at oxygenating and ventilating the patient were met with extreme resistance and difficulty-an airway situation that could have been catastrophic if not for quick reaction maneuvers performed that would ultimately go on to remedy the issue at hand. We hope to raise awareness regarding the airway challenges posed by GPA and delve into its management as a means of improving recognition and preparing clinicians to treat this condition.
Keywords: acute rheumatology; difficult airway management; diffuse alveolar hemorrhage; granulomatosis with polyangiitis (gpa); intubation complication; intubation response; pediatric respiratory diseases; pediatric respiratory failure; pediatrics emergency; wegener's disease.
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