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Asthma Associated With Incontinentia Pigmenti and Fanconi AnemiaAsthma Without Cellular Inflammation: Variable Airflow Limitation Without Cellular Bronchitis

Angira Dasgupta, MD; May S. Sanaee, MD; Carla M. T. Bauer, PhD; Fernando M. Botelho, PhD; Donald M. Arnold, MD; Martin R. Stampfli, PhD; Parameswaran Nair, MD, PhD, FCCP
Author and Funding Information

From the Divisions of Respirology (Drs Dasgupta, Sanaee, Stampfli, and Nair) and Hematology (Dr Arnold), Department of Medicine; and McMaster Immunology Research Centre Department of Pathology and Molecular Medicine (Drs Bauer, Botelho, and Stampfli), McMaster University, Hamilton, ON, Canada.

Correspondence to: Parameswaran Nair, MD, PhD, FCCP, Firestone Institute for Respiratory Health, St. Joseph’s Healthcare, 50 Charlton Ave E, Hamilton, ON, L8N 4A6, Canada; e-mail: parames@mcmaster.ca


Funding/Support: Dr Nair is supported by a Canada Research Chair in Airway Inflammometry. Dr Stampfli is supported by an operating grant from the Canadian Institutes of Health Research. Dr Arnold is supported by a New Investigator Award from the Canadian Institutes of Health Research in partnership with F. Hoffmann-La Roche Ltd.

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


Chest. 2013;143(3):856-858. doi:10.1378/chest.12-0859
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Airway inflammation is considered a central component of asthma and, therefore, international guidelines recommend antiinflammatory medications. We describe the clinical history of a 34-year-old woman with airway hyperresponsiveness and asthma who had a reduced ability to mount an inflammatory response due to two unrelated and rare genetic conditions: Fanconi anemia and incontinentia pigmenti. Absence of eosinophils in blood and sputum led to a successful reduction in the dose of corticosteroids without loss of asthma control demonstrating the clinical utility of monitoring treatment using biomarkers and the importance of recognizing the components of airway diseases that contribute to symptoms.


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