Abstract

A previously well 15-year-old boy presented to the emergency room with a three-month history of intermittent epistaxis, hemoptysis, intermittent low-grade fevers, cough and weight loss. The history began three months before presentation; while he had been participating in ultimate fighting, he was punched in the nose and developed a significant episode of epistaxis. Following this, he developed several more episodes of epistaxis and hemoptysis requiring cautery on three occasions in a community emergency department. During this period, a chest x-ray was performed that was reported as normal. Two months before admission, he developed right-sided otalgia, pharyngitis, and facial pain and tenderness. A computed tomography of his sinuses showed right maxillary sinusitis. A computed tomography of the chest was reported as normal. One month before presentation, his cough worsened. He developed dyspnea, hoarseness and gingival bleeding. The patient’s family doctor had referred him to a local otolaryngologist who had performed sinus lavage, septoplasty for septal perforation and placed tympanostomy tubes 10 days before admission. On review of his systems, the patient disclosed orthopnea, past arthralgia in the right thumb interphalangeal joint and right knee, vertigo and anorexia. He believed that the majority of his 14 kg weight loss had occurred over the past month. He denied any other sites of bleeding, recent travel or sick contacts. His medical history was significant for a two-year history of intermittent recurrent sinusitis treated with antibiotics, multiple tympanostomy tubes for frequent childhood otitis media and mild asthma. His family history was unremarkable. His physical examination revealed an unwell, but nontoxic-appearing boy, who was lying in bed (with the head of the bed tilted at 45 degrees) and breathing shallowly and sweating. His vital signs revealed a temperature of 36.8°C, heart rate of 90 beats/min, respiratory rate of 18 breaths/min, blood pressure of 120/70 mmHg and an oxygen saturation of 95% on room air. His weight was 64 kg, and his height was 172 cm. Head and neck examination revealed friable and bloody gums, and bloody crusts in the nares, but was otherwise normal. On respiratory examination, he had laboured breathing and breathlessness with hoarse speech, inspiratory and expiratory stridor, decreased air entry to the bases of the lungs bilaterally and a prolonged expiratory phase. No wheezes or crackles were audible. The remainder of the examination was unremarkable. On later consultation with the rheumatology consultant, he was found to have small joint effusions of the right thumb interphalangeal joint and the right knee. Initial laboratory studies included a normal complete blood count, electrolytes, blood urea nitrogen, creatinine and coagulation screen. The erythrocyte sedimentation rate was elevated at 40 mm/h. Additional imaging and bloodwork revealed the diagnosis.

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