(1193) Sealing the Deal: Endobronchial Valves in a Pediatric Patient With Acute Lupus Pneumonitis

Introduction: Acute lupus pneumonitis (ALP) is a rare initial presentation of systemic lupus erythematosus (SLE), which can progress to complicated respiratory failure. This includes bronchopleural fistulas (BPFs) with persistent air leak, which can be difficult to manage in patients requiring invasive mechanical ventilation. Endobronchial valves (EBVs) can be safely used as a therapy to manage BPFs with persistent air leak in intubated pediatric patients with ALP.

Description: A previously healthy 15 year old female presented to the pediatric intensive care unit in respiratory failure after one week of worsening cough and fevers. She was found to have multiorgan injury and subsequently diagnosed with SLE and ALP. Her respiratory failure was complicated by necrotizing pneumonia secondary to Methicillin-Sensitive Staphylococcus aureus and superimposed Aspergillus fumigatus. She developed persistent tension pneumothoraces, and subsequent imaging showed BPFs for which healing was impeded by high-dose steroids and immunosuppressants needed for SLE. Interventional pulmonology placed three EBVs in the right upper and middle lobes with resolution of her tension pneumothoraces, though a small air leak persisted. Despite initial improvement, she struggled with poor oxygenation and venovenous extracorporeal membrane oxygenation (VV ECMO) was utilized, allowing for additional healing. Three days after cannulation onto VV ECMO, she was extubated and respiratory support was quickly weaned with resolution of the air leak. She began rehabilitation, after 44 days was decannulated, and after 137 days in the hospital she walked out without respiratory support. Her EBVs were removed 6 months after discharge.

Discussion: ALP is a rare and possibly life-threatening complication of SLE which may be misdiagnosed on initial presentation, as it can range in severity. This can delay treatment of SLE and cause additional complications such as persistent BPFs. EBVs are one-way valves placed via bronchoscopy that can be a safe method to improve or prevent air leak and allow BPFs and lung parenchyma to heal in pediatric patients. The use of EBVs allows for a less invasive option in the management of BPFs, has increasing prevalence in pediatrics, and should be considered for pediatric patients with persistent BPFs.