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Images in medicine
Pulmonary alveolar proteinosis
treatment by whole-lung lavage
Pulmonary alveolar proteinosis (PAP) is a rare condition which is
characterised by the abnormal accumulation of proteinaceous
material in the alveolar spaces, with resulting impairment in
oxygen exchange across the involved alveoli. The diagnosis of
PAP can be established by the classic ‘milky’ effluent bronchoalveolar lavage fluid (BALF). The current effective treatment
for PAP is whole-lung lavage (WLL). We offer one case of
PAP with apparent presentations and the clinical course. This
report provides some information about the diagnosis and
treatment of PAP (see PAP video online).
A 39-year-old man, an ex-smoker, was brought to our respiratory department due to dry cough and progressive exertional
dyspnoea for 7 months. He had been an antimonial worker for
6 years. Physical examination was significant for perioral
cyanosis, mild clubbing and diffuse rales. Arterial blood gas
analysis on room air showed oxygen partial pressure (PaO2)
71.5 mm Hg. The serum lactate dehydrogenase level was
elevated at 301 U/l (normal range: 135e225 U/l). The pulmonary function test exhibited moderate restriction and severe
decrement in diffusing capacity. The total lung capacity was
3.43 l (60.1% pred) and diffusing capacity of lung for carbon
monoxide (DLco) was 3.32 mmol/min/kPa (36.5% pred). Serial
chest high resolution computerised tomography (HRCT)
revealed progressive bilateral ground glass opacities consistent
with ‘crazy paving’ configuration, extending but sparing some
areas (figure 1A,B). The bronchoscopy showed normal endobronchial anatomy. Histological examination of the trans-
bronchial lung biopsy showed chronic inflammation, which was
negative for PAP. But the BALF revealed a turbid fluid. BALF light
microscopy revealed periodic acid-schiff positive amorphous
proteinaceous material. Electron microscopy showed intra-alveolar debris containing lamellar bodies (figure 2AeD). The
definitive diagnosis of PAP was made upon the pathognomonic
HRCT appearance and BALF examination.
After comprehensive consultation with specialists and the
family, therapeutic WLL was performed first on the left lung.
Under general anaesthesia, the two lungs were separated by
double-lumen endobronchial tube. The left lung was flooded
with 378C warm saline, with manual chest percussion.1 A total
of 10e15 cycles and about 15 l saline was used for each lung. The
right lung was lavaged 1 week later. The patient was kept
haemodynamically stable and there was no hypoxaemia during
the lavage procedure; oxygen saturation can rise up to 100%
sometimes. After both lungs lavage, the PaO2 on room air
increased up to 86.5 mm Hg, the DLco improved to 53.6% pred
and HRCT findings showed apparent clearing of the lungs
(figure 1C,D).
Following discharge, this patient was free of respiratory
symptoms and there was no indication for further lavage. In
view of the rare occurrence, long-term follow-up is necessary.2
The aetiology and pathogenesis of PAP remain unclear. The
suspected causes are associated with occupational exposures,
which may result in impaired alveolar macrophage function
and surfactant clearance.1 Progressive dyspnoea and dry cough
are common symptoms. The diagnosis can be established by
the classic milky BALF, and lung biopsy is not the diagnostic
gold standard. WLL is the current mainstay of treatment for
PAP. The minority can be spontaneous cure, but there are no
simple biochemical parameters to predict the prognosis (see
supplementary material online).
Figure 1 Changes in serial chest HRCT image features. Images (A, B) during 8 months without any therapy showed a strong worsening of diffuse
ground-glass opacification and interlobular septa thickening, a characteristic ‘crazy paving’. Image C after left whole-lung lavage (WLL) and D after right
WLL. Note the marked clearing of the infiltrates in both lungs after lavage.
CaiCopyright
C, Ye M, Xu H, Article
et al. Postgrad
Med J (or
(2012).
doi:10.1136/postgradmedj-2011-130620
author
their
employer) 2012. Produced
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Images in medicine
Figure 2 Lung histopathology, bronchoalveolar lavage fluid (BALF) appearance and cytology, and ultrastructure. (A). Transbronchial biopsy appeared
as chronic inflammation (3100). (B). Cytospin of BALF showed alveolar macrophages containing abundant PAS-positive material in a background of
PAS-positive granular lipoproteinaceous material (3400). (C). BALF appearance from whole-lung lavage (up) and bronchoscopic lavage (lower). Note
the ‘milky’ effluent. (D). Ultrastructure of BALF showing cellular debris and concentrically laminated phospholipid lamellar bodies (320 000). PAS,
periodic acid-schiff stain.
Chang Cai, Min Ye, Honglei Xu, Yuping Li
approval of the version to be published. All authors have read and approved the final
manuscript. All authors participated in determining the order of authorship.
Division of Pulmonary Medicine, First Affiliated Hospital of Wenzhou Medical College,
Wenzhou, China
Competing interests None.
Patient consent Obtained.
Correspondence to Yuping Li, Division of Pulmonary Medicine, First Affiliated
Hospital of Wenzhou Medical College, Fuxue Lane No. 2, Wenzhou 32500, China;
[email protected]
Ethics approval Approval provided by the Hospital Committee of Medical Research
Ethics.
< Additional materials are published online only. To view these files please visit the
Provenance and peer review Not commissioned; externally peer reviewed.
journal online (http://pmj.bmj.com).
Data sharing statement Dr Chang Cai has included details of data sharing in the box
provided. Data available on request from the corresponding author: Yuping Li. Email:
[email protected]. The initial data were deposited in the First Affiliated Hospital of
Wenzhou Medical College Medical records system.
Contributors CC has contributed to conception and design, and carried out the
manuscript preparation. MY and HX have carried out acquisition and interpretation of
data. YL has revised it critically for important intellectual content and provided final
Learning points
< Pulmonary alveolar proteinosis (PAP) is a rare condition,
which can be diagnosed by the characteristic chest HRCT
appearances and classic ‘milky’ bronchoalveolar lavage fluid
findings.
< Whole-lung lavage can be considered to be a safe and
effective treatment for PAP.
This paper is freely available online under the BMJ Journals unlocked scheme, see
http://pmj.bmj.com/site/about/unlocked.xhtml
Postgrad Med J 2012;-:1e2. doi:10.1136/postgradmedj-2011-130620
REFERENCES
1.
2.
Seymour JF, Presneill JJ. Pulmonary alveolar proteinosis progress in the first 44
years. Am J Respir Crit Care Med 2002;166:215e35.
Shah PL, Hansell D, Lawson PR, et al. Pulmonary alveolar proteinosis: clinical aspects
and current concepts on pathogenesis. Thorax 2000;55:67e77.
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Cai C, Ye M, Xu H, et al. Postgrad Med J (2012). doi:10.1136/postgradmedj-2011-130620
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Pulmonary alveolar proteinosis treatment by
whole-lung lavage
Chang Cai, Min Ye, Honglei Xu and Yuping Li
Postgrad Med J published online April 29, 2012
Updated information and services can be found at:
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620
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Supplementary Supplementary material can be found at:
Material http://pmj.bmj.com/content/suppl/2012/04/28/postgradmedj-2011-130
620.DC1
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