Diffuse alveolar hemorrhage (DAH) is a life-threatening pulmonary emergency that may occur as a manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We report a rare case of perinuclear antineutrophil cytoplasmic antibody (p-ANCA)-positive vasculitis presenting with severe DAH without renal involvement, preceded by acute bilateral hearing loss. A previously healthy 21-year-old woman presented with hemoptysis, severe hypoxemia, and respiratory failure requiring mechanical ventilation. Chest imaging showed bilateral diffuse infiltrates and ground-glass opacities. Bronchoalveolar lavage findings were consistent with DAH. Serologic workup revealed positive p-ANCA with negative cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) and anti-glomerular basement membrane (anti-GBM) antibodies. Renal function and urinalysis remained normal throughout admission. The patient was treated with pulse intravenous methylprednisolone followed by oral corticosteroids, leading to rapid clinical improvement and successful extubation within five days. Further history revealed bilateral hearing loss one week before presentation, which may represent a possible early otologic manifestation of vasculitis. This case highlights that ANCA-associated vasculitis may present as isolated DAH without renal involvement, while unexplained hearing loss may provide an early clue to underlying AAV before the onset of life-threatening pulmonary manifestations. Early recognition and prompt immunosuppressive therapy are essential to improve outcomes.
Alhejaili et al. (Tue,) studied this question.
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