Tuberc Respir Dis.  2009 Dec;67(6):569-573.

A Case of Idiopathic Pulmonary Alveolar Proteinosis Treated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) after Partial Response to Whole Lung Lavage

• Song JW
• Park SH
• Kang KW

Abstract

Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by surfactant component accumulation in the alveolar space. Idiopathic PAP has recently been recognized as a autoimmune disease of impaired alveolar macrophage function caused by autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF). While whole lung lavage has been the standard treatment, not every patient shows a complete response. Subcutaneous injection or inhalation of GM-CSF is another promising treatment option for PAP. A 45-year-old patient visited our hospital for dyspnea, he was diagnosed as PAP and underwent whole lung lavage. Eighteen months later, the patient had not achieved complete remission in despite of initial response. After then he was administered with GM-CSF (5 microgram/kg/day, subcutaneous injection) for fivetimes a week during 2 months. Nine months later, the abnormal shadows in high-resolution computed tomography (HRCT) decreased and the patient fully recovered in forced vital capacity. After 60 months, the HRCT scan showed complete remission of PAP.

Keyword

Pulmonary Alveolar Proteinosis; Granulocyte-Macrophage Colony-Stimulating Factor, Bronchoalveolar Lavage

MeSH Terms

Autoantibodies
Autoimmune Diseases
Bronchoalveolar Lavage
Colony-Stimulating Factors
Dyspnea
Granulocyte-Macrophage Colony-Stimulating Factor
Humans
Inhalation
Injections, Subcutaneous
Lung
Macrophages, Alveolar
Middle Aged
Pulmonary Alveolar Proteinosis
Vital Capacity
Autoantibodies
Colony-Stimulating Factors
Granulocyte-Macrophage Colony-Stimulating Factor

Figure

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  Figure 1 Initial pre-whole lung lavage chest radiograph and high-resolution computed tomography (HRCT) findings. Simple chest X-ray shows diffuse ground glass opacities on both middle and lower lung field (A). HRCT scan shows ground glass opacities and interlobular septal thickening, a pattern commonly characterized as "crazy paving" (B).

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  Figure 2 Whole lung lavage fluid. The retrived fluid of the left lung in the first bottle is more milky and turbid. The turbidity and amount of sediment decreased gradually.

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  Figure 3 Serial follow up high-resolution computed tomography (HRCT) findings. Pre GM-CSF treatment baseline HRCT scan shows diffuse extent of "crazy paving" pattern (A). Post-9 months follow up HRCT scan shows some improvement in extent of lesion (B). Post-14 months follow up HRCT scan shows marked improvement in disease extent and intensity of ground glass opacities (C). Post-60 months follow up HRCT scans shows no more unusual opacities (D).

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