Whole lung lavage therapy (WLL) of pulmonary alveolar proteinosis (PAP): A global survey of current practices and procedures

Campo, Ilaria; Luisetti, Maurizio; Griese, Matthias; Trapnell, Bruce C.; Bonella, Francesco; Grutters, Jan C.; Nakata, Koh; Moorsel, Coline van; Costabel, Ulrich; Cottin, Vincent; Ichiwata, Toshio; Inoue, Yoshikazu; Braschi, Antonio; Bonizzoni, Giacomo; Iotti, Giorgio; Tinelli, Carmine; Rodi, Giuseppe

doi:10.1183/13993003.congress-2016.pa3871

Cited by 4 publications

(8 citation statements)

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“…WLL was found as the sufficient treatment for PAP patients due to significant improvement of spirometric parameters, oxygen saturation, arterial blood gas analysis and 6MWD after WLL (p-value < 0.0001). This confirmed previous findings indicating that WLL is the sufficient treatment for PAP [3,11,[13][14][15].…”

Section: Discussionsupporting

confidence: 92%

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An Eleven-Year Retrospective Cross-Sectional Study on Pulmonary Alveolar Proteinosis

Kiani

Parsa

Naghan

et al. 2018

Advances in Respiratory Medicine

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Section: Discussionsupporting

confidence: 92%

“…Whole-lung lavage (WLL), which is known as the gold standard treatment [7][8][9][10][11][12] for PAP, was used for the first time among Iranian PAP patients in the present study. Since 1958, more than 500 cases of PAP have been reported worldwide [1].…”

Section: Introductionmentioning

confidence: 99%

An Eleven-Year Retrospective Cross-Sectional Study on Pulmonary Alveolar Proteinosis

Kiani

Parsa

Naghan

et al. 2018

Advances in Respiratory Medicine

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“…The primary indications for WLL include decreased pulmonary function, decreased arterial oxygen saturation at rest, and abnormal radiographic findings. All of these were present in this case [16]. Complications that may occur during WLL are fever, hypoxemia, pneumonia, and pneumothorax, all of which were absent in this case.…”

Section: Discussionmentioning

confidence: 50%

“…However, in most institutions, lavage is performed under general anesthesia with lung isolation [17]. There are several variations considering the anesthetic maintenance agent, the choice of lung to be lavaged, the patient's position at the time of lavage, the volume of lavage fluid, and the method of chest percussion [16].…”

Section: Discussionmentioning

confidence: 99%

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Whole lung lavage using a rapid infusion system to treat a patient with pulmonary alveolar proteinosis

Won

Park

Lee

et al. 2020

Yeungnam Univ J Med

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Whole lung lavage (WLL) is a therapeutic procedure to remove accumulated material by infusing and draining the lungs with lavage fluid. This procedure has been regarded as the current standard of care to treat pulmonary alveolar proteinosis. However, the WLL protocol has not yet been standardized and the technique has been refined and modified a number of times. A rapid infusion system is a device used to infuse blood or other fluids at precise rates and normothermic conditions. This device is not typically used in WLL, which relies on the passive infusion of fluids using the gravitational force. However, in this study we performed WLL using a rapid infusion system, since we aimed to take advantage of its shorter operation time and greater degree of control over fluid volume and temperature. The patient's symptoms improved without the occurrence of any complications.

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Pulmonary Alveolar Proteinosis Syndrome

Kelly

McCarthy

2020

Semin Respir Crit Care Med

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Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by progressive accumulation of pulmonary surfactant. This results in dyspnea, secondary pulmonary and systemic infection, and in some cases respiratory failure. PAP syndrome occurs in distinct diseases, classified according to pathogenetic mechanism; these include primary PAP (due to disruption of granulocyte-macrophage colony-stimulating factor [GM-CSF] signaling), secondary PAP (due to reduction in alveolar macrophage numbers/functions), and congenital PAP (due to disruption of surfactant production). In primary PAP, the most common cause is autoimmune PAP, which accounts for over 90% of all PAP syndrome. The pathogenesis is driven by reduced GM-CSF-signaling causing abnormal alveolar macrophage function which subsequently results in impaired alveolar surfactant clearance. Autoimmune PAP can be accurately diagnosed by serum GM-CSF autoantibody levels and there now exist other diagnostic tests for rare causes of PAP syndrome. The current standard treatment is whole lung lavage; however, there is emerging evidence to support the use of novel therapeutic approaches, including inhaled GM-CSF, immune modulation, gene and cell therapy, and targeting macrophage cholesterol homeostasis. Furthermore, several innovative approaches to monitor disease severity and response to therapy have recently been developed.

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Whole lung lavage therapy (WLL) of pulmonary alveolar proteinosis (PAP): A global survey of current practices and procedures

Cited by 4 publications

References 0 publications

An Eleven-Year Retrospective Cross-Sectional Study on Pulmonary Alveolar Proteinosis

An Eleven-Year Retrospective Cross-Sectional Study on Pulmonary Alveolar Proteinosis

Whole lung lavage using a rapid infusion system to treat a patient with pulmonary alveolar proteinosis

Pulmonary Alveolar Proteinosis Syndrome

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