Uncommon pulmonary presentation of IgG 4-related disease in a 15-year-old boy

Abstract

IgG 4-related disease was fi rst described in adults with autoimmune pancreatitis but is now known to affect multiple organs. Lung involvement has never been described in children to our knowledge. Here, we report an adolescent presenting with recurrent dry cough and hemoptysis who was found to have venous ectasia in the left upper lobe, and diffuse bronchiectasis. Sustained high levels of IgG 4 (1,090 mg/dL) were found, and the endobronchial biopsy revealed a marked infi ltration of plasma cells producing IgG 4 (ratio of IgG 4 plasma cells to IgG plasma cells > 50%). This unique case highlights the occurrence of IgG 4-related disease in a child and underscores the importance of careful scrutiny of all investigations in complex pediatric respiratory cases. © 2013 American College of Chest Physicians.