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Uncommon Pulmonary Presentation of IgG4-Related Disease in a 15-Year-Old BoyLung Presentation of IgG4-Related Disease in a Boy

Massimo Pifferi, MD, PhD; Maria Di Cicco, MD; Andrew Bush, MD; Davide Caramella, MD; Marco Chilosi, MD; Attilio L. Boner, MD
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From the Department of Pediatrics (Drs Pifferi and Di Cicco) and the Department of Diagnostic and Interventional Radiology (Dr Caramella), University of Pisa, Pisa, Italy; the Department of Pathology and Diagnostics (Dr Chilosi) and the Department of Pediatrics (Dr Boner), University of Verona, Verona, Italy; and the Imperial School of Medicine at the National Heart and Lung Institute (Dr Bush), London, England.

Correspondence to: Massimo Pifferi, MD, PhD, Department of Pediatrics, University of Pisa, Via Roma 67 56126 Pisa, Italy; e-mail: m.pifferi@med.unipi.it


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2013;144(2):669-671. doi:10.1378/chest.12-3088
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IgG4-related disease was first 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 IgG4 (1,090 mg/dL) were found, and the endobronchial biopsy revealed a marked infiltration of plasma cells producing IgG4 (ratio of IgG4 plasma cells to IgG plasma cells >50%). This unique case highlights the occurrence of IgG4-related disease in a child and underscores the importance of careful scrutiny of all investigations in complex pediatric respiratory cases.

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