A Global Survey on Whole Lung Lavage in Pulmonary Alveolar Proteinosis

Campo, Ilaria; Luisetti, Maurizio; Griese, Matthias; Trapnell, Bruce C.; Bonella, Francesco; Grutters, Jan C.; Nakata, Koh; Moorsel, Coline H.M. van; Costabel, Ulrich; Cottin, Vincent; Ichiwata, Toshio; Inoue, Yoshikazu; Braschi, Antonio; Bonizzoni, Giacomo; Iotti, Giorgio; Tinelli, Carmine; Rodi, Giuseppe; Arai, Toru; Bazhanov, Andrey; Ben-Dov, Issahar; Casey, Alicia; Doğru, Deniz; Gesierich, Wolfgang; Halme, Maija; Henry, Michael R.; Herth, Felix J.F.; Wang, Huiying; Ilkovich, Julia; Irani, Sarosh; Kolek, Vı́tězslav; Morais, António; Morgan, Cliff; Nicolaï, Thomas; Novikova, Lubov; Primhak, Robert; Reiter, Karl; Retsch‐Bogart, George; Russi, Eric; Schmitz, Jochen; Schön, Carola; Schümann, Christian; Veltkamp, Marcel; Verwey, Charl; Wood, Robert E.

doi:10.1016/j.chest.2016.04.030

Cited by 19 publications

(14 citation statements)

References 5 publications

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“…In a recent international multicenter survey involving 20 centers worldwide [19,20], general anesthesia was used almost universally in adults with a double-lumen tracheal tube in 2 consecutive sessions (1 lung per session), with an interval of 1-2 weeks between WLL procedures observed by 50% of centers. The amount of fluid used to perform the WLL varied also greatly (800 mL of warm saline on average), with great variability of the total volume used per lung, with a range from 5 to 40 L. Most (14 of 20) centers used chest percussion to emulsify the PAP sediment to improve therapeutic efficiency.…”

Section: Discussionmentioning

confidence: 99%

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Efficacy of Whole-Lung Lavage in Pulmonary Alveolar Proteinosis: A Multicenter International Study of GELF

Gay¹,

Wallaert

Nowak³

et al. 2017

Respiration

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Background: New therapies have emerged in the treatment of pulmonary alveolar proteinosis (PAP) and, therefore, there is a real need to evaluate the efficacy of whole-lung lavage (WLL) in this rare disease. Objectives: The aim of this study was to assess the efficacy of WLL in patients with PAP. Methods: We included 33 patients from 12 centers, which are members of the French-Speaking Thoracic Endoscopy Group, for analysis. Data collection concerned patients and disease characteristics, pulmonary function tests (PFTs) and technical information on the procedure. Results: The median age of the patients was 44 years (range 13-77). There were 23 (71.9%) patients with respiratory insufficiency at presentation. All patients underwent WLL by general anesthesia and selective lung ventilation, except 1 who underwent awake flexible bronchoscopy. We noted differences in the technique, as 12 (36.36%) patients had percussion during the procedure and only 4 (12.1%) patients underwent 2-lung lavage during 1 anesthesia. A median of 12 L was used to perform WLL (1.0-40 L). Complications occurred in 11 (33.3%) patients, and 18 (56.25%) of them relapsed in a median period of 16.9 months. No significant changes were found in any PFT parameters studied, except for PaO₂, which was significantly improved by 6.375 mm Hg (p = 0.0213) after the procedure compared to before. Conclusions: Although the application of the WLL technique was variable, overall, it significantly improved patients' short-term respiratory condition by improving PaO₂. However, a long-term effect needs to be confirmed, as many of our patients relapsed.

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Section: Discussionmentioning

confidence: 99%

“…The authors finally concluded that WLL is safe and effective as a therapy for PAP. This study represents a first step in developing an evidence-based, best-practice approach to standardize WLL therapy for PAP [19,20]. …”

Section: Discussionmentioning

confidence: 99%

Efficacy of Whole-Lung Lavage in Pulmonary Alveolar Proteinosis: A Multicenter International Study of GELF

Gay¹,

Wallaert

Nowak³

et al. 2017

Respiration

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“…[82][83][84] Despite its widespread use, the procedure is not standardized and there is a lack of clarity regarding both the indications and the procedural method, including the volume to be infused, use of mechanical percussion, and how to evaluate its efficacy. 85,86 The usual end point for termination of WLL is an improvement in visual clearing of the lavage effluent. While, to date, no studies have systematically evaluated the procedure, its utilization in clinical practice, or the efficacy or durability of clinical responses, it is widely accepted to improve symptoms, radiographic findings, and oxygenation in patients.…”

Section: Therapeutic Approaches Whole-lung Lavagementioning

confidence: 99%

“…85,87,88 The procedure remains highly operator-dependent and is generally indicated when respiratory symptoms impair the quality of life, oxygen treatment is required, or lung function deviates from normal. 2,[5][6][7]86 In one report with adequate data for 231 individuals, WLL was associated with an increase in overall 5-year survival ( 2 and most patients had a clinical response; however, approximately 5% of the cases failed to respond. The median duration of benefit, or time to next lavage, was 15 months, and only 20% of the patients remained free of recurrence at 3 years.…”

Section: Therapeutic Approaches Whole-lung Lavagementioning

confidence: 99%

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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“…The gold standard of treatment is the whole lung lavage (WLL), even if this procedure is not yet standardized [6,7] and is associated with a very variable response rate [8,9]. WLL physically removes the accumulated surfactant, however in some patients it is ineffective, despite repeated WLL treatments.…”

Section: Introductionmentioning

confidence: 99%

Inhaled GM-CSF in a Pulmonary Alveolar Proteinosis Patient Refractory to Plasmapheresis Combined with Multiple Whole Lung Lavages

Campo¹,

Mariani²,

Paracchini³

et al. 2017

Arch Pulmonol Respir Care

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A autoimmune Pulmonary Alveolar Proteinosis (PAP) patient with persistent disease underwent 3 Whole Lung Lavages (WLLs), 10 plasmapheresis sessions and further 3 WLL, from October 2004 to May 2007. Nevertheless HRTC and pulmonary function test (PFT) showed a persistent residual disease of mild degree. At the beginning of 2010, the patient was admitted to inhaled rGM-CSF (Sargramostim) therapy as compassionate treatment. GM-CSF was administered by Akita 2 nebulizer (Vectura), as follow: 250 mcg/ day every other week for 12 weeks, then 250 mcg/day on 2 consecutive days every 2 weeks for 6 months. Follow up visits were scheduled at 3, 10, 18, 30 months and after that once a year. Functional and HRCT data and PaO2 were collected. Since the start of the inhaled GM-CSF therapy, the patient no more required WLL. Furthermore we found a signifi cant increase in DLCO% (p=0.013) and FVC% (p=0.023) while %FEV1 show a positive trend. No substantial differences in blood gas analysis. The pulmonary involvement at HRCT shows a signifi cant decrease of lung infi ltrates (p=0.039) in terms of pathological segments. These data underscore the utility of inhaled GM-CSF not only in case of progressing disease but also in case of refractory patients with persistent lung infi ltrates, in order to increase the response rate.

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A Global Survey on Whole Lung Lavage in Pulmonary Alveolar Proteinosis

Cited by 19 publications

References 5 publications

Efficacy of Whole-Lung Lavage in Pulmonary Alveolar Proteinosis: A Multicenter International Study of GELF

Efficacy of Whole-Lung Lavage in Pulmonary Alveolar Proteinosis: A Multicenter International Study of GELF

Pulmonary Alveolar Proteinosis Syndrome

Inhaled GM-CSF in a Pulmonary Alveolar Proteinosis Patient Refractory to Plasmapheresis Combined with Multiple Whole Lung Lavages

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