6533b837fe1ef96bd12a1fbc

RESEARCH PRODUCT

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

P. BenesR. E. SchopfFerlinz RV. SchulzJ. Müller-quernheim

subject

AdultMalePathologymedicine.medical_specialtyPhagocytosisOpportunistic InfectionsPulmonary Alveolar ProteinosisPathogenesisPhagocytosisDrug DiscoverymedicineMacrophageHumansMacrophage Migration-Inhibitory FactorsGenetics (clinical)Lungmedicine.diagnostic_testbiologyMacrophagesfood and beveragesProteinsGeneral MedicineMacrophage Activationmedicine.diseaseRespiratory burstMolecular WeightPulmonary AlveoliBronchoalveolar lavagemedicine.anatomical_structureImmunologyLuminescent Measurementsbiology.proteinMolecular MedicineAntibodyPulmonary alveolar proteinosisEnergy MetabolismBronchoalveolar Lavage Fluid

description

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.

10.1007/bf01745499https://pubmed.ncbi.nlm.nih.gov/3323640