Chest
Clinical InvestigationsPulmonary Alveolar Proteinosis: Staining for Surfactant Apoprotein in Alveolar Proteinosis and in Conditions Simulating It
Section snippets
Materials and Methods
Formalin fixed paraffin-embedded tissue sections from patients with proven alveolar proteinosis were studied in 13 cases. Sections from blocks of lavage material were examined in another four patients. The diagnosis in all of these 17 patients had been established by open lung biopsy specimen, and the patients were successfully treated by pulmonary lavage. Tissues from five patients with intra-alveolar accumulation of PAS positive material, recognized at autopsy, associated with hematologic
Primary Alveolar Proteinosis
The intra-alveolar material stained uniformly with rabbit anti-monkey surfactant apoprotein specific IgG (SSIgG) (Fig 1). In areas where the apoprotein material was completely filling the alveoli, the staining was usually uniform; the unstained parts usually consisted of blank spaces left due to crystals, presumably cholesterol or lipid droplets. When a sizable fragment of the tissue was available, it was apparent that the accumulation of alveolar proteinosis material was not uniform.
Discussion
The two main points to be discussed in the present study are the following: (a) diagnostic specificity of PAS staining and of immunoperoxidase staining for surfactant apoprotein, to differentiate between primary and secondary pulmonary alveolar proteinosis; and (b) the information about the pathogenesis of the disease.
Staining by the PAS Method vs Immunoperoxidase Staining for Surfactant Apoprotein
Strong granular staining by the PAS method has been considered to be diagnostic of the disease even though PAS positivity is not specific for the alveolar contents of pulmonary alveolar proteinosis. It has been shown that the alveolar contents in pulmonary alveolar proteinosis are, in part, sufactant or surfactant derived.4, 5, 6, 7, 8, 9, 10, 11, 12 Thus, a specific staining method for surfactant should provide a more specific diagnostic test. This logical assumption appears to be borne out by
Pathogenesis
Surfactant, under physiologic conditions, appears to be cleared by (a) re-uptake by alveolar cells; (b) centripetal movement and along the mucociliary escalator; (c) lymphatics; and (d) macrophages.8, 15, 16, 17, 18, 19 In theory, excessive accumulation of surfactant could occur either due to excessive production or deficient removal or both.20 Earlier studies indicate that the rate of synthesis of surfactant in patients with alveolar proteinosis is not increased.21 Even though hypertrophy and
Acknowledgments
The authors are grateful to Carole Mallick for technical assistance, Linda Shab for photography, and Carolyn Karpa for typing the manuscript. The authors are also thankful to the following for their kind cooperation in providing some of the tissue blocks used in this study: Charles Kuhn, MD; Jacqueline J. Coalson, PhD; Raymond Maguire, MD; SJC Miller, MD; EG McKee, MD; Donald J Jarzynski, MD; RS Golembiewski, MD; Stanley Weitzner, MD; Alfred M. Shideler, MD; Steven C. Bauserman, MD; Harold E.
References (29)
- et al.
Management of pulmonary alveolar proteinosis
Chest
(1980) - et al.
Bronchopulmonary fluids in pulmonary alveolar proteinosis
J Allerg Clin Immunol
(1973) - et al.
On the origin of the membranous intra-alveolar material in pulmonary alveolar proteinosis
Exp Mol Pathol
(1974) Pulmonary alveolar proteinosis: abnormal in vitro function of alveolar macrophages
Chest
(1979)- et al.
Analysis of pulmonary surfactant apoproteins by electrophoresis
Biochim Biophys Acta
(1981) - et al.
Pulmonary alveolar proteinosis
N Engl J Med
(1958) - et al.
Pulmonary alveolar proteinosis: the diagnosis by segmental lavage
Am Rev Respir Dis
(1980) - et al.
Diagnosis and management of alveolar proteinosis: the role of electronmicroscopy
Thorax
(1975) - et al.
Pulmonary alveolar proteinosis
Am J Clin Pathol
(1967) - et al.
Lipids found in pulmonary lavage of patients with alveolar proteinosis and in rabbit lung lamellar organelles
Am Rev Respir Dis
(1976)
Pulmonary alveolar proteinosis: analysis of airway and alveolar proteins
Am Rev Respir Dis
Characterization of a high-molecular-weight glycoprotein isolated from the pulmonary secretions of patients with alveolar proteinosis
Biochem J
Surfactant apoprotein in nonmalignant pulmonary disorders
Am J Pathol
Demonstration of surface active material in human lung lavage effluent, abstracted
Clin Res
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Manuscript received June 7; revision accepted July 30