P2.108: A Case of Air Leak Syndrome and Diffuse Alveolar Hemorrhage in an Adolescent Male with Granulomatosis with Polyangiitis

Introduction: Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis characterized by aberrant neutrophil activation resulting in endothelial damage, classically affecting the upper and lower respiratory tract and kidneys. Pediatric cases are rare, with an estimated 1.8 cases per 1 million person-years in the United States. We present a case of GPA with delayed diagnosis and subsequent severe systemic disease requiring treatment in the pediatric intensive care unit (PICU).

Case Description: A 13-year-old male with a 10-month history of recurrent sinusitis and epistaxis presented to the emergency room for hemoptysis which began 3 days after hospital discharge for sepsis secondary to severe coagulase-negative staphylococcal otitis externa. He had acute hypoxemic respiratory failure with chest x-ray (CXR) showing diffuse interstitial opacities concerning for diffuse alveolar hemorrhage (DAH). He was admitted to PICU on bilevel positive airway pressure ventilation but required intubation and mechanical ventilation for worsening hypoxemia from severe acute respiratory distress syndrome (ARDS) with an oxygenation index of 17.9 by hospital day (HD) 2. He developed air leak syndrome (ALS) on HD 4 with CXR showing pneumomediastinum and subcutaneous emphysema. Due to continued hypoxemia with ALS, he was escalated to high frequency oscillatory ventilation on HD 6 and had bilateral chest tubes placed on HD 7.

Rheumatology was consulted for suspicion of vasculitis. GPA was confirmed with patient’s symptoms, positive antineutrophil cytoplasmic antibody and proteinase-3 antibody. He was treated with high dose steroids and plasmapheresis followed by rituximab with gradually downtrending inflammatory markers. He was extubated on HD 31 but developed a spontaneous pneumothorax on HD 47 that resolved after chest tube placement. He was eventually discharged to inpatient rehabilitation.

Discussion: Air leak syndrome, characterized by the presence of air outside of the airways and alveoli, is caused by disruption of the bronchovascular interface from alveolar injury in ARDS, barotrauma or volutrauma from mechanical ventilation, pulmonary infection, or trauma. The extensive alveolar injury and rupture from this patient’s severe GPA directly led to ALS through DAH, subsequent ARDS, and the need for high-pressure mechanical ventilation and predisposed him to his subsequent pneumothorax. To our knowledge, this is among the first cases to document ALS in a pediatric patient with GPA complicated by DAH. A review of 61 case reports of GPA revealed that while 36% of patients with DAH and ARDS required extracorporeal membrane oxygenation, there were no reported instances of air leak in patients with DAH. This case underscores the importance of recognizing ALS as a potential but severe complication of systemic GPA.

Conclusion: This case highlights how the severe systemic manifestations of GPA and resultant treatment can predispose patients to develop air leak syndrome. Awareness and early recognition of GPA with appropriate treatment may prevent the development of severe systemic disease and complications.