AR TIC LESC ASES COURSESQUIZ

AboutBlogDonate
SEARCH

Radiopaedia is free thanks to our Supporters & Advertisers

Pulmonary emphysema
Dr Henry Knipe and Dr Luke Danaher et al.

Pulmonary emphysema is defined as the "abnormal permanent enlargement of the airspaces distal to the
terminal bronchioles accompanied by destruction of the alveolar wall and without obvious fibrosis".
Emphysema is one of the entities grouped together as chronic obstructive pulmonary disease. Emphysema
is best evaluated on CT, although indirect signs can be noticed on conventional radiography in a proportion
of cases. This article focuses on panlobular emphysema, paraseptal emphysema, and in particular
centrilobular emphysema.

Epidemiology
At the time of initial writing, approximately 210 million people are affected worldwide leading to 3 million
deaths annually 1. It is predominantly a disease of middle to late life owing to the cumulative effect of
smoking and other environmental risk factors. It traditionally affected more men than women but with
increased smoking and environmental risk factor exposure among women, the incidence is now equal
between the sexes. Patients with genetic risk factors such as alpha-1-antitrypsin deficiency may present
earlier according to phenotype.

Risk factors
smoking: by far the most common
alpha-1-antitrypsin (AAT) deficiency
intravenous injection of methylphenidate (Ritalin lung)

Clinical presentation
The clinical features of emphysema should be distinguished from the signs and symptoms of chronic
bronchitis. Patients with emphysema are hypocapnoeic and are often referred to as "pink puffers". This
compares with the hypercapnoea and cyanosis of chronic bronchitis with patients referred to as "blue
bloaters". In practice, features of these two syndromes coexist as chronic obstructive pulmonary disease.

Patients typically report dyspnoea without significant sputum production.

Signs of emphysema include:

tachypnoea
absence of cyanosis
pursed-lip breathing
chest hyperinflation
reduced breath sounds
hyper-resonant to percussion
cor pulmonale (late)

Pathology
 Emphysema is one of a heterogeneous group of pathological processes forming chronic obstructive
pulmonary disease, and is itself a relatively vague term encompassing a number of entities and
morphological patterns including:

morphologic subtypes
o centrilobular emphysema (most common)
o panlobular emphysema
o paraseptal emphysema
o paracicatricial emphysema
o localised emphysema
idiopathic giant bullous emphysema (or vanishing lung syndrome)
congenital lobar emphysema
pulmonary interstitial emphysema
The three morphologic subtypes of emphysema are named according to their relationship to the secondary
pulmonary lobule.

Centrilobular emphysema is the most frequently encountered type and affects the proximal respiratory
bronchioles, particularly of the upper zones. It is strongly associated with smoking in a dose-dependent
way 3. Rarely, severe centrilobular emphysema can be seen in the bases in patients with Salla disease4.

Panlobular emphysema in contrast, affects the entire secondary pulmonary lobule and is more
pronounced in the lower zones, matching areas of maximal blood flow. It is seen particularly in alpha-1-
antitrypsin (AAT) deficiency (exacerbated by smoking) 2-4, intravenous injection of methylphenidate (Ritalin
lung) 3 or Swyer-James syndrome 4.

Paraseptal emphysema affects the peripheral parts of the secondary pulmonary lobule, and is usually
located adjacent to the pleural surfaces (including pleural fissures) 3. It is also associated with smoking, and
can lead to the formation of subpleural bullae and spontaneous pneumothorax 3.

Radiographic features
Plain radiograph
Except in the case of very advanced disease with bulla formation, chest radiography does not image
emphysema directly, but rather infers the diagnosis due to associated features 2-3:

hyperinflation
o flattened hemidiaphragm(s): most reliable sign
o increased and usually irregular radiolucency of the lungs
o increased retrosternal airspace
o increased antero-posterior diameter of chest
o widely spaced ribs
o sternal bowing
o tenting of the diaphragm
o saber-sheath trachea
vascular changes
o paucity of blood vessels, often distorted
o pulmonary arterial hypertension
pruning of peripheral vessels
 increased calibre of central arteries
right ventricular enlargement
It should be remembered, however, that the most common plain film appearance of COPD is "normal" and
the role of chest radiography is to eliminate other causes of lung symptoms such as infection,
bronchiectasis or cancer 6.

CT
CT is currently the modality of choice for detecting emphysema; HRCT is particularly effective. It should be
noted, however, that there is relatively poor correlation between autopsy-proven emphysema, pulmonary
function test abnormalities and CT with 20% of pathology-proven cases not being evident on CT and 40%
of patients with abnormal CT having normal pulmonary function tests.

CT is able to discriminate between centrilobular, panlobular, and paraseptal emphysema.

Centrilobular emphysema
Centrilobular is by far the most common type encountered, and is a common finding in asymptomatic
elderly patients. It is predominantly located in the upper zones of each lobe (i.e. apical and posterior
segments of the upper lobes, and superior segment of the lower lobes) and has a patchy distribution 4. It
appears as focal lucencies (emphysematous spaces) which measure up to 1 cm in diameter, located
centrally within the secondary pulmonary lobule, often with a central or peripheral dot representing the
central bronchovascular bundle 2-4.

Panlobular emphysema
Panlobular emphysema is predominantly located in the lower lobes, has a uniform distribution across parts
of the secondary pulmonary lobule, which are homogeneously reduced in attenuation 2-4.

Paraseptal emphysema
Paraseptal emphysema is located adjacent to the pleura and septal lines with a peripheral distribution
within the secondary pulmonary lobule. The affected lobules are almost always subpleural, and
demonstrate small focal lucencies up to 10 mm in size.

Any lucency >10 mm should be referred to as subpleural blebs/bullae (synonymous) 3.

In all three subtypes, the emphysematous spaces are not bounded by any visible wall 3.

MRI
MRI is in the research phases for evaluation of lung parenchymal abnormalities like emphysema. Dynamic
breathing MRI may have a future role in assessing pulmonary emphysema. 5

Treatment and prognosis

smoking cessation
oxygen therapy (in chronic hypoxaemia)
symptom and exacerbation control
o short and long-acting beta-2 agonists
o inhaled anticholinergics
o inhaled glucocorticoids
o antibiotics
 pulmonary rehabilitation
In patients with severe bullous change with resultant compression of remaning normal lung
parenchyma, lung volume reduction therapy may be considered in selected patients.

Lung transplantation is considered in cases of alpha-1-antitrypsin deficiency.

Prognosis is worse in patients who continue to smoke, are alpha-1-antitrypsin deficient, have low FEV 1at
time of diagnosis, or have other comorbidities (e.g. heart failure, respiratory failure, frequent
exacerbations).

Differential diagnosis
cystic lung disease: all have visible walls
o lymphangiomyomatosis (LAM)
o pulmonary Langerhans cell histiocytosis (LCH): often co-exists with emphysema
o honeycomb lung: usually reduced lung volumes
References
1. Kemp SV, Polkey MI, Shah PL. The epidemiology, etiology, clinical features, and natural history
of emphysema. Thorac Surg Clin. 2009;19 (2): 149-58. doi:10.1016/[Link].2009.03.003 - Pubmed citation
2. Robertson RJ. Imaging in the evaluation of emphysema. Thorax. 1999;54 (5): 379. Thorax
(link) - Free text at pubmed - Pubmed citation
3. Collins J, Stern EJ. Chest radiology, the essentials. Lippincott Williams & Wilkins. (2007)
ISBN:0781763142. Read it at Google Books - Find it at Amazon
4. Stern EJ, Frank MS. CT of the lung in patients with pulmonary emphysema: diagnosis,
quantification, and correlation with pathologic and physiologic findings. AJR Am J Roentgenol. 1994;162 (4):
791-8. AJR Am J Roentgenol (abstract) - Pubmed citation
5. Suga K, Tsukuda T, Awaya H et-al. Impaired respiratory mechanics in pulmonary emphysema:
evaluation with dynamic breathing MRI. J Magn Reson Imaging. 1999;10 (4): 510-20. J Magn Reson Imaging
(link) - Pubmed citation
6. Takasugi JE, Godwin JD. Radiology of chronic obstructive pulmonary disease. Radiol. Clin.
North Am. 1998;36 (1): 29-55. - Pubmed citation
7. Foster WL, Gimenez EI, Roubidoux MA et-al. The emphysemas: radiologic-pathologic
correlations. Radiographics. 1993;13 (2): 311-28. doi:10.1148/radiographics.13.2.8460222 - Pubmed citation

8. Robbins & Cotran Pathologic Basis of Disease: Expert Consult - Online: Expert Consult -
Online. Not Avail. ISBN:0323296351. Read it at Google Books - Find it at Amazon