Pulmonary Alveolar Proteinosis

doi:10.1059/0003-4819-62-2-292

Pulmonary Alveolar Proteinosis

Report of Six Cases, Review of the Literature, and Formulation of a New Theory

ROGER K. LARSON, M.D., F.A.C.P.; and
RICHARD GORDINIER, M.D.

Requests for reprints should be addressed to Roger K. Larson, M.D., Fresno County General Hospital,
445 S. Cedar Ave., Fresno, Calif
.

Excerpt

Beginning with the initial description by Rosen, Castleman, and Liebow (1), a total of 79 cases of pulmonary alveolar proteinosis has been recorded in the medical literature (1-51). It is the purpose of this paper to add 6 new cases, to summarize the clinical features of all 85 cases, and to formulate a new theory about the origin of the intra-alveolar material that is so characteristic of the disease. Eighty-four of these 85 cases were confirmed by histological examination. One diagnosis was based on sputum examination only (25).

CASE STUDIES CASE 1

W. H., a 38-year-old Caucasian male police officer,

Acknowledgments

The authors are grateful to John Murray, M.D., and the Department of Medicine, University of California Medical Center, Los Angeles, for providing Cases 1 and 2; Malcolm Masten, M.D., Fresno, for Case 3; Henry Rosenstiel, M.D., Chief of Medicine, Fresno Veterans Administration Hospital, for Case 5; and Byron Evans, M.D., Fresno, for Case 6.

We would also like to express our appreciation to John Miller, M.D., Chief of Pathology, Fresno General Hospital, for reviewing the histology in these cases; H. Corwin Hinshaw, M.D., Clinical Professor of Medicine, University of California, San Francisco; and John F. Murray, M.D., Associate Professor of Medicine, University of California, Los Angeles, for their helpful comments in the preparation of this manuscript.

Summario in Interlingua

Es revistate 85 casos de proteinosis pulmonoalveolari, incluse sex que es nove. Plure importante characteristicas clinic es confirmate e re-sublineate secundo lor merito. Superimponite infectiones pulmonar es frequente e hasardose. Le extense varietate de organismos pathogene—particularmente fungos—que ha essite isolate ab iste patientes rende evidente le importantia de studios cultural pro le selection del tractamento. Therapias specific contra le infectiones e methodos non-specific visante a promover le elimination del materiales alveolari ha succedite a tener vive le majoritate del patientes. Le uso de steroides adrenal pare esser contraindicate.

Es proponite un nove theoria pathogenetic. Le similitude es signalate que existe inter le material de replenation alveolari in proteinosis pulmono-alveolari e le agentes de activitate al superficie que es normalmente secernite per le cellulas alveolo-epithelial. Certe morbos—per exemplo le syndrome de anxietate respiratori de neonatos—ha essite attribuite a un carentia in le mentionate material de activitate al superficie. Per analogia con altere conditiones, il pare plausibile supponer que un morbo etiam existe que es associate con hypersecretion. Es postulate le possibilitate que proteinosis pulmono-alveolar es un tal morbo. Ben que iste thoria non es capace a promover le clarification del etiologia del morbo, illo va forsan servir le utile function de inspirar e guidar futur investigationes.

Article and Author Information

From the Department of Medicine, Fresno County General Hospital, Fresno, Calif., and the Department of Medicine, University of California Medical Center, Los Angeles, Calif.
- Received August 4, 1964.
- Accepted November 10, 1964.