[Pulmonary alveolar proteinosis: a disease caused by surfactant accumulation, and new treatment with sargramostim] Nederlands tijdschrift voor geneeskunde [Ned Tijdschr Geneeskd] Journal article | | Title | [Pulmonary alveolar proteinosis: a disease caused by surfactant accumulation, and new treatment with sargramostim] | | Author(s) | Huisman P, Grutters JC, van den Bosch JM | | Institution | Ziekenhuis Amstelland, afd. Longziekten en Tuberculose, Postbus 328, 1180 AH Amstelveen. petra.huisman@planet.nl | | Source | Ned Tijdschr Geneeskd 2008 Jun 28; 152(26):1450-4. | | MeSH | Adult
Autoantibodies
Bronchoalveolar Lavage
Bronchoalveolar Lavage Fluid
Female
Granulocyte Macrophage Colony-Stimulating Factors, Recombinant
Granulocyte-Macrophage Colony-Stimulating Factor
Humans
Immunoglobulin G
Male
Middle Aged
Pregnancy
Pregnancy Complications
Pulmonary Alveolar Proteinosis
Treatment Outcome
| | Abstract | 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. | | Language | dut | | Pub Type(s) | Case Reports
English Abstract
Journal Article
| | PubMed ID | 18666660 |
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