Pulmonary Hemorrhage Due to Granulomatosis with Polyangiitis in a Pediatric Patient

ABSTRACT

INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a rare rheumatologic disease in pediatric patients but with a similar presentation and organ involvement as an adult patient. We present a case of a patient with pulmonary hemorrhage due to GPA who was managed without extracorporeal life support. DESCRIPTION A 14 year-old female with no medical history presented with migratory polyarthritis, sore throat, chest tightness, fatigue, and positive rheumatoid factor. Found to be hypoxic on presentation. Her respiratory failure progressed, requiring intubation and inhaled nitric oxide, with minimal improvement. A chest CT showed nonspecific bilateral multifocal, patchy airspace opacification. Her C-ANCA and proteinase 3 antibody were positive, making GPA the most likely diagnosis for which she was started on methylprednisolone and rituximab. Her hypoxemia continued to worsen despite maximal mechanical ventilator support and neuromuscular blockade infusion. She had bloody secretions from her endotracheal tube, concerning for pulmonary hemorrhage, despite high positive end-expiratory pressure. A chest radiograph at that time was consistent with worsening bilateral infiltrates. Echocardiogram showed normal biventricular function, pulmonary hypertension, and a 1.8 cm by 1.5 cm thrombus at the cavoatrial junction. A multidisciplinary team determined that the location of the clot precluded placement of ECMO cannulas without risking clot mobilization. Plasmapheresis was emergently initiated followed by further rituximab and cyclophosphamide. Her respiratory status stabilized after being placed in the prone position. She was ultimately discharged home after a prolonged period of intubation and hospitalization, on a steroid taper, oral anticoagulation for her cavoatrial thrombus, and maintenance rituximab therapy. DISCUSSION: This case highlights a rare case of GPA with multiorgan involvement in a pediatric patient resulting in refractory hypoxemia treated with aggressive rheumatologic therapy and proning. There is limited evidence for the efficacy of plasmapheresis in patients with GPA, although this patient may have benefited from it since she was not safe for extracorporeal life support. Furthermore, as highlighted through the COVID-19 pandemic, proning proved crucial in managing her severe hypoxemia.