D27-15 Case Presentation: Giant Cell Interstitial Pneumonia Presenting as Diffuse Alveolar Hemorrhage

Abstract Introduction Giant cell interstitial pneumonia (GCIP) is a rare form of interstitial lung disease, seldom described in pediatric patients, often associated with exposure to heavy metals. Case Description 11 yo previously healthy female presented with shortness of breath (SOB) who developed acute respiratory failure with pulmonary hemorrhage, renal and cardiac failure. Remained intubated for 8 days, treated with albuterol, prednisone (40mg BID) and antibiotics. Infectious and rheumatological workups were negative. She was discharged home on steroid taper; however, during the next several months, she had multiple ED visits for SOB and was unable to wean below 20mg of prednisone. 7 months following initial encounter, she was readmitted due to persistent SOB. Workup notable for depressed LV function and chest CT with mild ground glass opacities in the lung bases. The patient was discharged on low dose daily steroids. In the interim she developed influenza with worsening hemoptysis which improved with increased prednisone. Triple endoscopy was completed 1 month after illness; ENT and GI evaluations were normal; however, bronchoscopy was suggestive of ongoing pulmonary hemorrhage. Given the cardiac dysfunction, a cardiac catheterization was done and a small LUL vessel was coiled. She continued to experience small amounts of hemoptysis daily. Her steroids were stopped due to concern that cardiac dysfunction was secondary to chronic daily steroid use. However, her respiratory symptoms and hemoptysis worsened.A repeat HRCT showed persistent mild ground glass opacities bilaterally and decision to proceed with a lung biopsy was made. Histology showed GCIP. After discussion, the father stated he worked with heavy metals on the property but did not believe that the patient was exposed. However, the pathology was consistent with heavy metal accumulation in macrophages/giant cells. Energy dispersive spectroscopy was done to identify the metal component, however it was unsuccessful. The patient was admitted for a 3-day burst of methylprednisolone (1g/day) with close monitoring. A bronchoscopy was completed 3 months following treatment and showed no evidence of hemorrhage. Discussion GCIP, a rare form of interstitial lung disease is mostly seen in adults with prior heavy metal exposure presenting as pulmonary fibrosis. GCIP is exceptionally rare in children. Treatment is often high dose steroids either as a steroid burst with a taper or pulse dose steroids. Although our patient was initially treated with prednisone 40mg BID x 2 weeks and a taper it was ultimately the pulse dose steroids (1g x 3d) that led to disease control. This abstract is funded by: None