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Pulmonary Alveolar Proteinosis


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Measurement of GM-CSF autoantibody

Pulmonary Alveolar Proteinosis

Treatments for Pulmonary Alveolar Proteinosis (PAP)

Standard therapy

Whole lung lavage under general anesthesia

全身麻酔下の全肺洗浄法Using a double-lumen intratracheal tube, more than 20L of saline is infused into each lung to remove the accumulated surfactant, while mechanical ventilation is applied to the other lung. Although safe in the majority of cases, the highly invasive nature of the procedure remains a concern, especially in patients with severe disease.


Bronchoscopic lavage

Bronchoscopic segmental lavage is repeated using 500-1000 ml of saline as a safe alternative in patients with severe hypoxemia.

Treatment for secondary PAP

Secondary PAP can be improved through treatment of the underlying diseases in some cases. Whole lung lavage may improve non-resolving secondary PAP, but its efficacy remains unclear.

A case of PAP treated with whole lung lavage

A 50 year old man complaining of persistent cough visited his family physician. Ground glass opacities in both lower lung fields were demonstrated on chest X-ray. Administration of antibiotics had no effect and he was temporally diagnosed with interstitial pneumonia. Bronchoscopy was performed but only provided limited data and a diagnosis could not be reached due to severe coughing during the procedure. As hypoxemia progressed, corticosteroids were administered but resulted in no improvement. He was referred to a hospital where the diagnosis of PAP was established. He underwent whole lung lavage which successfully improved the disease.

全肺洗浄例

GM-CSF inhalation

Method

Treatments include high-dose GM-CSF administration (125 -150 mcg twice daily on days 1-8, none on days 9-14) for six 2-week cycles followed by low-dose administration (125-150 mcg once daily on days 1-4, none on days 5-14) for six 2-week cycles. 125-150 mcg of lyophilized Leukine dissolved in 2 ml of sterile saline is inhaled as an aqueous aerosol using a LC-Plus or LC-Star nebulizer with a manual, interrupter valve connected to a PARI Turbo BOY compressor (PARI GmbH, Starnberg, Germany) (ref, 6).

Efficacy

When a clinical response is defined as a reduction in A-aDO2 by at least 10 torr at the end of treatment compared to baseline, the response rate has been reported to be 62% (ref. 6).

Advantages

・No need to be hospitalized.
・The duration is 20-30 minutes for each daily inhalation at home.

Disadvantages

Clinical studies of GM-CSF inhalation

PaO2, AaDO2, and %VC were improved after inhalation therapy.

Alveolar-arterial oxygen difference (A-aDO2) was measured to determine the response to inhaled GM-CSF in patients with autoimmune PAP. Figure shows overall A-aDO2 (mean ±SE) for participants receiving inhalation therapy with GM-CSF (p < 0.05 (asterisk) and < 0.001 (double asterisk)). Thirty-nine patients completed the high-dose induction therapy period (weeks 1, 12, and 24, n=39) and 35 patients completed subsequent low-dose maintenance therapy period (weeks 36, n=35) (ref. 6).


Changes in cell numbers and GM-CSF antibody concentration of bronchoalveolar lavage fluid (BALF).

After inhalation therapy, cell numbers were increased, while GM-CSF antibody concentrations were decreased in BALF samples obtained from the three cases of our pilot study (ref. 7).


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