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A macrophage-suppressing 40-kD protein in a case of pulmonary alveolar proteinosis

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Summary

Pulmonary alveolar proteinosis (PAP) is a rare disease of unknown etiology. Macrophage dysfunctions are claimed to be involved in the pathogenesis. We investigated phagocytosis and oxidative metabolism of alveolar macrophages in a case of pulmonary alveolar proteinosis. These cells phagocytize normally and phagocytizable stimulants cause a normal oxidative burst. In response to the membrane signals phorbolmyristate acetate and aggregated immunoglobulin, however, no stimulated turnover of the oxidative metabolism can be observed. A 40-kD protein found in the lavage fluid mediates this macrophage-inhibiting effect. This phenomenon may contribute to the frequent opportunistic infections seen in PAP patients. It can be concluded from our data that the high frequency of infections with opportunistic species in these patients can be reduced by therapeutic bronchoalveolar lavage. By this procedure the abnormal macrophage-suppressing protein can be washed out of the lung at an early stage of the disease.

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Abbreviations

AM:

alveolar macrophage

BM:

blood monocyte

BAL:

bronchoalveolar lavage

CL:

chemiluminescence

Ig:

immunoglobulin

kD:

kilodalton

PAP:

pulmonary alveolar proteinosis

PMA:

phorbolmyristate acetate

SRBC:

sheep red blood cells

Z:

zymosan

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Müller-Quernheim, J., Schopf, R.E., Benes, P. et al. A macrophage-suppressing 40-kD protein in a case of pulmonary alveolar proteinosis. Klin Wochenschr 65, 893–897 (1987). https://doi.org/10.1007/BF01745499

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Key words

  • Pulmonary alveolar proteinosis
  • Alveolar macrophages
  • Oxidative metabolism