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Study Description

The purpose of this study is to (1) compare a technically improved assay with an existing assay used to measure serum anti-GM-CSF antibodies in stored serum samples previously obtained from patients diagnosed with either primary, secondary, congenital or idiopathic pulmonary alveolar proteinosis (PAP), other chronic diseases or disease-free, healthy individuals; (2) determine the prevalence and levels of anti-GM-CSF autoantibodies and (3) define the breadth of the autoimmune antibody responses in primary PAP patients from the United States, Japan, Australia, and Europe using previously collected serum samples; and (4) using a chart review approach, compare the clinical, radiologic and laboratory features of primary PAP patients to determine if differences exist among patients in these globally geographically distributed regions.

Authorized Access
Publicly Available Data (Public ftp)
Study Inclusion/Exclusion Criteria

Data and Serum Inclusion Criteria

  • Data and Serum must be from individuals of any age with confirmed diagnosis of PAP based on the following criteria:
    • Radiographic features consistent with PAP
    • Pathological or cytological findings consistent with PAP determined by either open lung biopsy, transbronchial biopsy or bronchoalveolar lavage cell/fluid cytology
    • Elevated anti-GM-CSF autoantibody concentration, when available
  • Ability of the international PI's and their research coordinators to ship samples in compliance with this study

Data and Serum Exclusion Criteria

  • No established diagnosis of PAP
  • Incomplete chart records in which >25% of the requested data is unable to be confirmed

Study History

  • Study Activated July 3, 2008
  • First Accrual July 3, 2008
  • Study Closed to Accrual in data analysis on January 13, 2015

Selected Publications
Diseases/Traits Related to Study (MeSH terms)
Links to Related Resources
Authorized Data Access Requests
Study Attribution
  • Study Chairs
    • Bruce Trapnell, MD. Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
  • Participating Site Principal Investigators
    • Koh Nakata, MD. University of Niigata Medical and Dental School, Niigata, Japan.
    • John Seymour, MB. Peter Mac Hospital, Melbourne, Australia.
    • Daniel Culver, MD. Cleveland Clinic Foundation, Cleveland, OH, USA.
    • James Ellis, MD. National Jewish Medical and Research Center, Denver, CO, USA.
    • Cliff Morgan, MD. Brompton Chest Hospital, London, UK.
    • Maurizio Luisetti, MD. Clinica Malattie Apparato Respiratorio, Universita di Pavia, Italy.
    • Mani Kavuru, MD. Brody School of Medicine Greenville, NC, USA.
    • Ulrich Costabel, MD. Ruhrlandklinik Essen, Essen, Germany.
    • Yoshikazu Inoue, MD. NHO Kinki-Chuo Chest Ctr.,Osaka, Japan.
    • Jeffrey Krischer, PhD. Data Management and Coordinating Center University of South Florida, Tampa, FL, USA.
  • Funding Source
    • National Institutes of Health, Bethesda, MD, USA.