Abstract P-294: ADOLESCENT WITH EHLERS DANLOS SYNDROME AND ACUTE PULMONARY HEMORRHAGE
Salas, A.1; Patel, A.2; Hart, L.2; Wen, A.1
1NYU Langone Medical Center, Pediatrics, New York, USA
2NYU School of Medicine, Pediatrics, New York, USA
Aims & Objectives:
Patients with Ehlers-Danlos syndrome (EDS) have abnormal collagen production or secretion leading to hyperextensibility of the skin, hypermobility of the joints, and increased tissue fragility. Although uncommon, respiratory manifestations of EDS have been described. The typical causes of death in patients with EDS are viscus rupture and arterial hemorrhage. Some deaths have been attributed to pulmonary hemorrhage. We report a case of acute pulmonary hemorrhage as the presenting finding of granulatomatosis with polyangiitis in a child with EDS.
A 14-year-old female with EDS presented with a 2-month history of fever, fatigue, unintentional weight loss, arthralgias, myalgias, and progressive dyspnea. Chest radiography revealed diffuse alveolar opacities and laboratory evaluation revealed anemia and thrombocytopenia. Chest computed tomography (CT) showed pulmonary hemorrhage. Anti-neutrophil cytoplasmic antibodies targeting proteinase 3 were positive for granulomatosis with polyangiitis. She was admitted on high-flow nasal cannula oxygen therapy and given pulse-dose intravenous methylprednisolone. Subsequent improvement in respiratory status was observed; she was treated with rituximab and weaned off oxygen. Patient was discharged home in stable condition on hospital day #5 and remained on oral prednisone, with a 4-week course of rituximab, and was started oral azathioprine.
To our knowledge this is the first reported case of polyangiitis with granulomatosis presenting with pulmonary hemorrhage in a child with EDS. An increased index of suspicion may improve outcomes.Copyright © 2017 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies