0
Editorials |

Therapeutic Whole Lung Lavage : A Stop-Gap Therapy for Alveolar Proteinosis

Mani S. Kavuru, MD, FCCP; Marc Popovich, MD
Author and Funding Information

Affiliations: Cleveland, OH
 ,  Dr. Kavuru is Director, Pulmonary Function Laboratory, Department of Pulmonary and Critical Care Medicine, and Dr. Popovich is on the staff of the Department of Anesthesiology, The Cleveland Clinic Foundation.

Correspondence to: Mani S. Kavuru, MD, FCCP, The Cleveland Clinic Foundation, 9500 Euclid Ave, Desk A90, Cleveland, OH 44195; e-mail: kavurum@ccf.org



Chest. 2002;122(4):1123-1124. doi:10.1378/chest.122.4.1123
Text Size: A A A
Published online

Extract

Pulmonary alveolar proteinosis (PAP) is characterized clinically by nonresolving pulmonary infiltrates and hypoxemia in patients in their third and fourth decades, and it occurs with a male predominance. This disease is quite remarkable for flooding of the alveoli with lipoproteinaceous material, yet with minimal local lung inflammation or distortion of architecture on biopsy, for reasons that are unknown. The last 8 years have seen some remarkable advances into the pathophysiology of this disease, through transgenic murine models that have clearly established that hematopoietic growth factor (granulocyte-macrophage colony stimulating factor [GM-CSF]) is critical for local regulation of surfactant homeostasis. Additional human studies have clearly linked the presence of a circulating, neutralizing anti–GM-CSF antibody in adults with idiopathic PAP.12 Therefore, in the current paradigm, there are three major clinical subtypes of PAP: the most common adult idiopathic variety, which is presumably autoimmune (with a circulating anti–GM-CSF antibody); a neonatal variety that is likely due to a defect in surfactant proteins B or C or the common β-chain of the GM-CSF receptor; and secondary PAP associated with occupational exposures or immunologic disorders. To date, there have been no reports of the presence of an antibody in the neonatal variety. Preliminary reports from several groups indicate that a subset of adult patients with idiopathic PAP respond favorably to GM-CSF therapy.34

First Page Preview

View Large
First page PDF preview

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Figures

Tables

References

NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Sign In to Access Full Content

MEMBER & INDIVIDUAL SUBSCRIBER

Want Access?

NEW TO CHEST?

Become a CHEST member and receive a FREE subscription as a benefit of membership.

Individuals can purchase this article on ScienceDirect.

Individuals can purchase a subscription to the journal.

Individuals can purchase a subscription to the journal or buy individual articles.

Learn more about membership or Purchase a Full Subscription.

INSTITUTIONAL ACCESS

Institutional access is now available through ScienceDirect and can be purchased at myelsevier.com.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543