Wash-out kinetics and efficacy of a modified lavage technique for alveolar proteinosis

Abstract

Whole lung lavage (WLL) is the standard treatment for pulmonary alveolar proteinosis (PAP). This study aimed to provide data about the kinetics of the protein wash-out, to identify factors influencing the protein concentration in the recovered fluid and to assess the efficacy of a modified lavage technique.Samples of 180 WLLs from 42 adult PAP patients were collected. 110 WLLs were performed according to the classical technique. In 70 WLLs, repeated manual ventilation was applied during the procedure. Spectrophotometry was used to measure the protein concentration in the recovered fluid.The initial protein concentration in the recovered fluid was 460 mg?dL -1 , the final concentration was 26 mg?dL -1 and the total amount of removed proteins during a lavage was 17.5 g. A history of dust exposure was associated with a higher residual protein concentration in the recovered fluid (p50.000013). The amount of removed proteins correlated inversely with the diffusing capacity of the lung for carbon monoxide (p50.001) and oxygen tension (p50.004). The modified technique removed a greater amount of proteins than the classical technique and prolonged the time to relapse (p50.011). Exposure to dust seems to influence the kinetics of the protein wash-out. Applying manual ventilation during the procedure can enhance the efficacy of WLL.

Pulmonale Alveolarproteinose

“…Am häufigsten sind Erwachsene zwischen dem 30. und 50. Lebensjahr betroffen [5][6][7], Männer 2-bis 3-mal häu-figer als Frauen [4][5][6][7][8], mit einer positiven Raucheranamnese in bis zu 80 % [4][5][6].…”

“…Eine Hypoxämie bei Belastung ist in etwa 50-70 % der Patienten zu sehen, in Ruhe nur in etwa einem Drittel der Patienten. Eine signifikante Steigerung der alveoloarteriellen Sauerstoffdruckdifferenz (AaDO2) wird als Indikator des Krankheitsschwergrads und als Kriterium für die Ganzlungenlavage betrachtet [6,9]. Ein gesteigerter Rechts-/Links-Shunt lässt sich ebenfalls feststellen.…”

“…Zunehmende Dyspnoe, Restriktion oder Gasaustauschstörung (insbesondere die Steigerung der AaDO2 nach Belastung) mit reduzierter körperlicher Belastbarkeit werden als Kriterien angewandt [6,7,25,28]. Nach dieser Behandlung kommen etwa ein bis zwei Drittel der Patienten in eine dauerhafte Remission [5,6,9].…”

“…In den meisten Fällen muss die Spü-lung mehrfach wiederholt werden, insbesondere bei den Rauchern [5]. Mehrere WLL-Modifikationen wurden beschrieben, um die Effektivität der Prozedur zu verbessern [4,6]. Bei der klassischen Technik, nach der Intubation mit einem Doppellumentubus, wird in Vollnarkose eine Atelektase induziert.…”

Rare (pulmonary) disease day: “feeding the breath, energy for life!”

“…Similarly, the estimated prevalence of lymphangioleiomyomatosis (LAM) in 2011 was one case per million people, while in 2013 the prevalence was reported to be 5.6 cases per million [6]. In the last 20 years, the knowledge of some rare pulmonary conditions has markedly improved and new therapies are now available that may be effective in changing the clinical course of several illness, such as PAH [7], chronic thromboembolic pulmonary hypertension [8][9][10][11][12], pulmonary vasculitis [13], bronchiolitis [14][15][16], LAM [17][18][19][20], pulmonary alveolar proteinosis [21,22], α 1 -antitrypsin deficiency [23], pulmonary Langerhans cell histiocytosis [24,25] and, more recently (and after several negative trials), idiopathic pulmonary fibrosis (IPF) [26][27][28][29][30]. New horizons of hope are now offered to patients suffering from rare pulmonary diseases.…”