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Original Research: INTERSTITIAL LUNG DISEASE |

A Kindred of Children With Interstitial Lung Disease*

Heather Thomas, MD; Kimberly A. Risma, MD, PhD; T. Brent Graham, MD; Alan S. Brody, MD; Gail H. Deutsch, MD; Lisa R. Young, MD; Patricia M. Joseph, MD, FCCP
Author and Funding Information

*From Cincinnati Children’s Hospital Medical Center, University of Cincinnati, Cincinnati, OH.

Correspondence to: Patricia Joseph, MD, FCCP, University of Cincinnati, 231 Albert Sabin Way, Cincinnati, OH 45267-0564; e-mail: patricia.joseph@uc.edu



Chest. 2007;132(1):221-230. doi:10.1378/chest.06-2476
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Background: Childhood interstitial lung disease (ILD) is a spectrum of diseases including many different rare lung conditions. We present a family with an unusual presentation of ILD in association with rheumatologic and immunologic abnormalities.

Methods: Eight children with a common father were evaluated for evidence of lung disease in association with rheumatologic findings. All underwent routine history and physical examination, hematologic evaluation, and chest radiography and/or CT scan of the chest. Seven children underwent a more extensive immunologic evaluation. Those who were able underwent pulmonary function testing, and four children underwent lung biopsy.

Results: Six of eight children with a common father were found to have radiographic findings consistent with ILD. These children also had evidence of autoimmune disease with joint symptoms, alopecia, rheumatoid factor production, and hypergammaglobulinemia. Open-lung biopsy in four children revealed a spectrum of pulmonary lymphoid proliferations ranging from reactive lymphoid hyperplasia to lymphoid interstitial pneumonia.

Conclusion: The findings of ILD and autoimmunity in a kindred of children suggest a novel genetic disorder of autosomal dominant pattern and variable penetrance. Although the precise pathogenesis remains unclear, these cases provide valuable insight into childhood ILD.

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