The Science Journal of the American Association for Respiratory Care

2010 OPEN FORUM Abstracts

WEGENER’S GRANULOMATOSIS SEEN IN AN ADOLESCENT FEMALE WITH BRONCHO-PLEURAL FISTULA

Gary R. Lowe1, Mark Heulitt2, Ariel Berlinski3; 1Respiratory Care/Nursing Research, Arkansas Children’s Hospital, Little Rock, AR; 2Department of Pediatrics, Critical Care Medicine, University of Arkansas for Medical Sciences, Little Rock, AR; 3Department of Pediatrics, Pulmonary Medicine, University of Arkansas for Medical Sciences, Little Rock, AR

Introduction: Diagnosis of Wegener’s Granulomatosis (WG) in an adolescent is challenging, and mortality is high in cases involving the pulmonary system. This case illustrates the presentation and course of a previously healthy 14 year old female ultimately diagnosed with WG, who developed long-term sequelae including broncho-pleural fistula (BPF). Case Summary: While being treated for a presumed pulmonary infection, this adolescent developed dyspnea and hemoptysis and presented for care at our institution. Her SpO2 was in the 60’s and CXR showed severe bilateral pneumonia. She received oxygen via .50 Venti-mask with improved oxygenation. She rapidly deteriorated, progressing from supplemental O2 to BiPap, ventilatory support, iNO, and ECMO over the next 73 hours. Her admitting diagnosis was respiratory failure and pneumonia. She required V-A ECMO following cardiac arrest. She remained on ECMO for 13 days, and weaned to conventional ventilation with iNO. In addition, she received PRBCs for anemia and substantial inotropic support. She developed persistent bilateral pneumothoraces and was diagnosed with BPF. She remained on ventilatory support for 25 days, and was successfully extubated. Autoimmune vasculitis was suspected and later confirmed with positive C-ANCA. Treatment for WG was initiated with steroids and cyclophosphamide. When the BPF did not resolve over time, she was discharged home with chest tube connected to a Heimlich valve. Discussion: WG is a rare autoimmune disease, characterized by the presence of necrotizing granulomas of the respiratory tract and/or kidneys. The overall prevalence is 1:20,000 to 1:30,000 for all ages and the peak incidence is ages 40-60. It is difficult to diagnose, requiring positive lab tests, CXR, and biopsy. This patient continues with BPF on the right side, requiring a chest tube with Heimlich valve. This case illustrates the rapid deterioration that could accompany WG and the importance of early recognition and intervention to treat the inflammatory process. It also illustrates that aggressive support, including ECMO, can lead to survival in patients with significant pulmonary involvement. Sponsored Research - None