NHLBI Working Group
Needs and Opportunities to Study Hypersensitivity Pneumonitis
Executive Summary

Full text of the published article is now available.

The National Heart, Lung, and Blood Institute in collaboration with the Office of Rare Diseases convened a Working Group on May 10-11, 2004, in Bethesda, Maryland to identify the opportunities for scientific advancement in Hypersensitivity Pneumonitis (HP). The Working Group participants discussed the current disease definition, methods to establish the diagnosis, occupational exposures, animal models, immunology, and host risk factors. It was recognized by the Working Group, that despite its putatively low prevalence, the impact on individuals of all ages continues to be a major health risk. Furthermore, the lack of recognition and limited understanding of the mechanisms of the disease have fostered development of chronic cases whom in general have been exposed to low levels of antigen for prolonged periods. Although removal from exposure may provide partial improvement, the overall treatment alternatives are limited. The group reviewed the current status of HP research and from these discussions provided the Institute with recommendations for new research opportunities to improve the accuracy of clinical diagnosis and investigate mechanisms.

The general recommendations of the Working Group are to:

• Establish a multi-center collaboration to enhance the understanding of the disease, including a tissue and radiographic repository to assist in the characterization of the disease.
• Define the natural history of the disease in the context of other lung diseases such as asthma and COPD. There is a need for better criteria for diagnosis.
• Use of circulating mononuclear cells or lymphocyte subsets isolated from peripheral blood in research may provide additional information to establish the diagnosis.
• Develop population-based studies, particularly in settings where the disease is endemic to provide additional insights for uniformity in environmental and clinical characterization of such cases.
• Use of minimally invasive biomarkers (e.g. nasal lavage, induced sputum, exhaled breath condensates, etc) in the diagnosis of the disease.
• Develop a battery of standardized antigens for diagnosis and make them available to clinicians.
• Use of quantitative and qualitative CT in prospective evaluation and longitudinal follow-up studies of HP and other organic dust diseases.
• Conduct genetic studies in the context of gene-environment interaction to better understand host risk factors.

Working Group Members

Chair: Jordan N. Fink, M.D., Medical College of Wisconsin

Members

• Yves F. Cormier, M.D., Hospital Laval, Canada
• Leland L. Fan, M.D., Texas Children's Hospital
• Teri J. Franks, M.D., Department of Pulmonary and Mediasyinal Pathology
• Kay Kreiss, M.D., National Institute for Occupational Safety and Health
• Steven Kunkel, Ph.D., University of Michigan Medical School
• David Lynch, M.D., MBBS, University of Colorado Health Science Center
• Santiago Quirce, M.D., Ph.D., Fundacion Jimenez Diaz, Alergia, Spain
• Cecil Rose, M.D., MPH, National Jewish Medical Research Center
• Robert Schleimer, Ph.D., Johns Hopkins University
• Mark R. Schuyler, M.D., VA Medical Center, Albuquerque, New Mexico
• Moises Selman, M.D., Inst. Nat. Enfermedades Resp., Mexico
• Douglas Trout, M.D., National Institute for Occupational Safety and Health
• Yasayuki Yoshizawa, M.D., Tokyo Dental and Medical University, Japan

NHLBI Staff

• Hector Ortega, M.D., Sc.D., Division of Lung Diseases
• Herbert Reynolds, M.D., Division of Lung Diseases

July 2004