Abstract Granulomatosis with polyangiitis (GPA) is classified as an antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis that typically affects patients aged 45-60. GPA can present with symptoms of sinusitis, hemoptysis, sensorineural hearing loss, and renal injury ranging from asymptomatic hematuria to acute renal failure. Diffuse alveolar hemorrhage (DAH), a condition characterized by hemoptysis that can lead to acute respiratory failure, is a severe complication of GPA that can be mitigated by prompt detection and treatment. In this case, a 40-year-old woman presented to the emergency department with six months of bilateral sinus pain, ear pain, and decreased hearing in her right ear that persisted despite several rounds of antibiotic therapy for presumed recurrent sinusitis. She was found to have imaging findings concerning for bilateral otomastoiditis as well as a new acute kidney injury (AKI). After evaluations by otolaryngology, infectious disease, and nephrology, she was deemed a non-surgical candidate, treated with daptomycin, and encouraged to increase oral intake for presumed dehydration-related AKI. During this time, patient began to have some hemoptysis with scant blood in sputum. After several days without clinical improvement on antibiotics and with worsening kidney dysfunction despite adequate oral intake, an autoimmune workup was initiated and rheumatology was consulted. Autoimmune labs were remarkable for a cytoplasmic-ANCA titer of 1:640. Patient also underwent a kidney biopsy that showed findings concerning for glomerulonephritis. She subsequently received the diagnosis of GPA and was soon started on high-dose methylprednisolone followed by plasma exchange therapy. Shortly after patient was initiated on steroids and plasma exchange, she had four episodes of hemoptysis within a few hours with associated dyspnea; during this time, she remained hemodynamically stable without hypoxia. Computed tomography (CT) scan with angiography of the chest revealed findings of bilateral centrilobular consolidations concerning for DAH. Patient was upgraded to the medical intensive care unit later that day for respiratory monitoring and was initiated on inhaled tranexamic acid. Her hemoptysis resolved without risk of respiratory compromise, and she was able to return to the medical floor for planned rituximab therapy after plasma exchange. This case illustrates the importance of early detection and treatment of DAH in a patient with GPA. Though DAH is a serious manifestation, continuing respiratory monitoring, management of hemoptysis with fibrinolytic agents, and treatment of GPA can mitigate risk for respiratory failure or hemodynamic collapse. This abstract is funded by: none
M Burin (Fri,) studied this question.
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