[Pulmonary alveolar proteinosis: a disease caused by surfactant accumulation, and new treatment with sargramostim].
The acquired form of pulmonary alveolar proteinosis was determined in 3 patients, a woman of 31 and 2 men of48 and 38 years, respectively. Their symptoms consisted of progressive dyspnoea, with or without coughing and a tight feeling in the chest. Bronchoscopy with bronchoalveolar lavage yielded milky white, frothy material, and high resolution CT revealed parenchymal densification. All 3 patients were successfully treated with recombinant granulocyte-macrophage colony-stimulating factor (GM-CSF; sargramostim); in addition, the first and last patient underwent total pulmonary lavage. During the pregnancy of the woman, the GM-CSF treatment was suspended; this was resumed after parturition, which took place via caesarean section. Pulmonary alveolar proteinosis is a rare disease characterised by accumulation of surfactant in the alveoli. Until recently, the treatment consisted only of total lung lavage under general anaesthesia. It has recently been discovered that IgG autoantibodies play an important role in the development ofthe disease, namely in the accumulation of surfactant in the alveoli. IgG autoantibodies appear to neutralise the biological activity of natural GM-CSF, which leads to accumulation of used surfactant in the alveoli and a decrease of the pulmonary diffusion capacity. These cases and other publications from the past few years underline the important role of GM-CSF, in addition to a total lung lavage, in the treatment of pulmonary alveolar proteinosis.
Huisman
P
P
Ziekenhuis Amstelland, afd. Longziekten en Tuberculose, Postbus 328, 1180 AH Amstelveen. petra.huisman@planet.nl
Grutters
J C
JC
van den Bosch
J M M
JM
D000328:Adult; D001323:Autoantibodies / Q000276:immunology; D018893:Bronchoalveolar Lavage / Q000379:methods; D001992:Bronchoalveolar Lavage Fluid; D005260:Female; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000378:metabolism / Q000627:therapeutic use*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008297:Male; D008875:Middle Aged; D011247:Pregnancy; D011248:Pregnancy Complications / Q000188:drug therapy*; D011649:Pulmonary Alveolar Proteinosis / Q000188:drug therapy* / Q000276:immunology* / Q000378:metabolism; D011994:Recombinant Proteins; D016896:Treatment Outcome
D001323:Autoantibodies; D007074:Immunoglobulin G; D011994:Recombinant Proteins; C081222:sargramostim; D016178:Granulocyte-Macrophage Colony-Stimulating Factor