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Interstitial Patterns

7
Nestor L. Müller and C. Isabela S. Silva

Many diseases involve predominantly or exclusively the SEPTAL PATTERN


pulmonary interstitium. The differential diagnosis of the
various entities on the chest radiograph and CT is based A septal pattern results from thickening of the interlobular
on the pattern and distribution of abnormalities and on septa (i.e., the tissue that separates the secondary pulmo-
the presence of associated findings, such as lymph node nary lobules) (Fig. 7-1). Normally, no septal lines can
enlargement or pleural effusion. Interstitial lung disease be identified on the radiograph, and only a few can be seen
results in five distinct radiologic patterns of abnormality: on high-resolution CT, mostly in the anterior and lower
septal, reticular, cystic, nodular, ground-glass opacities, aspects of the lower lobes. When thickened, interlobular
and consolidation. Although each of these patterns can be septa (septal lines) are seen on the radiograph as short (1-
seen on high-resolution CT and correlated with specific 2 cm) lines perpendicular to and continuous with the
histopathologic findings, the appearances on the chest pleura (Kerley’s B lines) or as longer (2-6 cm) lines oriented
radiograph are frequently nonspecific and sometimes mis- toward the hila (Kerley’s A lines) (Fig. 7-2). On high-resolu-
leading.1,2 A reticular pattern on the radiograph may result tion CT scan, septal lines can be seen as short lines that
from summation of smooth or irregular linear opacities, extend to the pleura in the lung periphery and as polygonal
cystic spaces, or both. In approximately 10% of patients arcades outlining one or more pulmonary lobules in more
with interstitial lung disease, the chest radiograph is central lung regions (see Fig. 7-2).5 Thickening of the inter-
normal.3 Numerous studies have shown that CT, particu- lobular septa may be caused by edema, cellular infiltration,
larly high-resolution CT, is superior to chest radiogra- or fibrosis. Septal thickening can be smooth, nodular, or
phy in the detection of parenchymal abnormalities and irregular in contour.
is more accurate in the differential diagnosis.1,2,4 High- The most common cause of a septal pattern as the
resolution CT is performed almost routinely in the evalu- predominant or only pattern is hydrostatic pulmonary
ation of patients with suspected or proven interstitial lung edema (Figs. 7-3 to 7-5).6 Less common causes of smooth
disease. septal thickening include lymphatic spread of carcinoma
Because of its low cost and low radiation dose, the (see Fig. 7-2),7 lymphoma (Fig. 7-6),8 leukemia,9 Churg-
chest radiograph remains the initial imaging modality of Strauss syndrome (Fig. 7-7),10 acute lung rejection (Fig.
choice in the evaluation of patients with suspected inter- 7-8),11 congenital lymphangiectasia,12 and Niemann-Pick
stitial lung disease and in the follow-up of these patients. syndrome (Fig. 7-9).13 These various conditions tend to
Serial chest radiographs often provide a clue to the correct result in extensive septal thickening that is usually bilat-
diagnosis by showing the course of the disease process. eral and often symmetric. Localized smooth septal thick-
In this chapter, we include the radiographic and the high- ening is frequently seen adjacent to pleural inflammation,
resolution CT patterns of abnormality, recognizing that particularly in patients with empyema or following
in a high percentage of cases accurate assessment of pleurodesis (Fig. 7-10),14 lymphoid interstitial pneumo-
presence, pattern, and extent of interstitial disease can nia,15 and idiopathic bronchiectasis.16 The interlobular
be made only on high-resolution CT. Consolidation may septal thickening in patients with bronchiectasis is pre-
occur in interstitial lung disease, but more commonly sumably due to impaired lymphatic drainage, and the
reflects the presence of airspace disease. Consolidation is extent of septal lines correlates with the extent and
discussed separately in Chapter 4. Text continued on p. 164.
158

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CHAPTER 7 ● Interstitial Patterns 159

■ FIGURE 7-1 Schematic drawing of septal pattern.


Schematic drawing of the right lung shows thin lines separated
by 1-2 cm that correspond to thickened interlobular septa.

A
■ FIGURE 7-2 Septal pattern in a patient with lymphangitic
carcinomatosis. A, Posteroanterior chest radiograph shows numerous
bilateral linear opacities. B, Magnified view of right lower lung zone shows
linear opacities (arrows) measuring 1-2 cm in length and perpendicular to
the pleura. These represent septal lines (Kerley’s B lines). B
Continued

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160 PART TWO ● Radiologic Manifestations of Lung Disease

■ FIGURE 7-2, cont’d C, High-resolution CT scan


shows 0.5-2 cm long lines (straight arrows) and polygonal
arcades outlining one or more pulmonary lobules. These
linear opacities (septal lines) reflect the presence of
thickening of the interlobular septa. Prominent
centrilobular dots (curved arrows), which represent
thickening of the interstitium along the centrilobular
bronchiole, and small left pleural effusion also are noted.
The patient was an 80-year-old man with lymphangitic
carcinomatosis.

■ FIGURE 7-3 Septal thickening resulting from


pulmonary edema. Histologic specimen shows
thickening of the interlobular septa and partial filling
of the airspaces by hydrostatic pulmonary
edema. (Courtesy of Dr. John English, Department of
Pathology, Vancouver General Hospital, Vancouver, Canada.)

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CHAPTER 7 ● Interstitial Patterns 161

A B
■ FIGURE 7-4 Septal pattern in a patient with interstitial pulmonary edema. A, Posteroanterior chest radiograph shows numerous bilateral linear
opacities. B, Magnified view of right lower lung zone shows linear opacities (arrows) measuring 1-2 cm in length and perpendicular to the pleura.
These represent septal (Kerley’s B) lines. Prominence of the pulmonary vessels and a small left pleural effusion also are noted. The patient was an
80-year-old woman with interstitial pulmonary edema as a result of left heart failure.

A B
■ FIGURE 7-5 Smooth septal thickening in a patient with interstitial pulmonary edema. A, High-resolution CT scan at the level of the lower lung
zones shows smooth septal lines perpendicular to the pleura (straight arrows) and more centrally as polygonal arcades (curved arrows). B, Coronal
reformation shows septal pattern mainly in the lower lung zones. The thickened septa are seen as smooth lines perpendicular to the pleura (straight
arrows) and as polygonal arcades (curved arrows). The patient was an 84-year-old woman with left heart failure and interstitial pulmonary edema.

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162 PART TWO ● Radiologic Manifestations of Lung Disease

A B
■ FIGURE 7-6 Septal thickening in lymphoma. A, Contrast-enhanced CT scan shows large anterior mediastinal mass. B, Lung windows show
bilateral thickening of the interlobular septa. The patient was a 35-year-old man with non-Hodgkin’s lymphoma.

■ FIGURE 7-7 Smooth septal thickening in Churg-Strauss syndrome.


High-resolution CT scan shows mild bilateral smooth thickening of several
interlobular septa (arrows). The patient was a 41-year-old man with
Churg-Strauss syndrome. The patient had normal cardiac function; the
interlobular septal thickening was due to eosinophilic infiltration.

A B
■ FIGURE 7-8 Smooth septal thickening in acute lung rejection. A, High-resolution CT scan performed on a multidetector scanner in a 41-year-old
man with unilateral left lung transplant shows thickening of several interlobular septa in the transplanted lung. Interstitial fibrosis, which was proven
to be nonspecific interstitial pneumonia, is evident in the native right lung. B, Coronal reformation shows extensive septal thickening in the
transplanted lung and lower lung zone fibrosis in the native lung. Transbronchial biopsy specimens of the left lung showed acute rejection.

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A B
■ FIGURE 7-9 Septal thickening in Niemann-Pick disease. A and B, High-resolution CT scans at the level of the bronchus intermedius (A) and
diaphragm (B) show mild bilateral interlobular septal thickening. The patient was a 43-year-old man with Niemann-Pick disease.

A B

C D
■ FIGURE 7-10 Septal thickening as a result of pleural inflammation. A and B, High-resolution CT scans at the level of inferior pulmonary veins (A)
and slightly caudal (B) show localized smooth septal thickening and parenchymal bands in the anterior region of the right middle lobe secondary to
chronic pleural inflammation. Note surgical resection of the anterior aspects of the right fourth, fifth, and sixth ribs. The patient was a 33-year-old
woman with prior resection of chest wall chondrosarcoma. C, High-resolution CT scan in another patient shows interlobular septal thickening in the
anterior region of the upper lobes. D, Contrast-enhanced CT scan photographed with soft tissue window shows increased soft tissue in the
parasternal region and bilateral chest wall and mediastinal pleural thickening. The patient was a 68-year-old man who developed sternal osteomyelitis
1 month after cardiopulmonary bypass graft. Sternal wound culture grew methicillin-resistant Staphylococcus aureus.
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164 PART TWO ● Radiologic Manifestations of Lung Disease

■ FIGURE 7-11 Septal thickening as a result of


lymphangitic carcinomatosis. Histologic specimen shows
nodular thickening of the interlobular septa by tumor
cells. (Courtesy of Dr. Julia Flint, Department of Pathology,
Vancouver General Hospital, Vancouver, Canada.)

■ FIGURE 7-12 Nodular septal thickening in


lymphangitic carcinomatosis. High-resolution CT scan
shows extensive bilateral septal thickening and small
left pleural effusion. Several of the thickened septa
have a beaded nodular appearance (arrows). The
patient was an 80-year-old man with lymphangitic
carcinomatosis. Also evident are enlarged mediastinal
lymph nodes.

severity of bronchiectasis.16 Nodular septal thickening mality seen in these patients. Other common findings
occurs most commonly in patients with lymphangitic car- include unilateral or asymmetric hilar lymph node enlarge-
cinomatosis (Figs. 7-11 and 7-12),7 sarcoidosis (Fig. 7-13),17 ment and pleural effusion. Although septal thickening may
silicosis, and coal workers’ pneumoconiosis.18,19 Less be seen in sarcoidosis, it is seldom the main finding. The
common causes include lymphoma,8 Kaposi’s sarcoma,20 nodules in sarcoidosis are typically most numerous along
leukemia,9 and amyloidosis.21 the bronchoarterial and pleural interstitium and along the
Patients with lymphangitic spread of tumor may have interlobar fissures (see Fig. 7-13).17,22
smooth or nodular septal thickening (see Figs. 7-2 and Irregular septal thickening is seen most commonly in
7-12).7 In most cases, septal thickening is the main abnor- patients with interstitial fibrosis, particularly idiopathic

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CHAPTER 7 ● Interstitial Patterns 165

■ FIGURE 7-13 Nodular septal thickening


in sarcoidosis. High-resolution CT scan shows
nodular thickening of the interlobular septa
(straight arrows). Other characteristic features
of sarcoidosis also are noted, including
nodules along the vessels (curved arrows) and
interlobar fissures (arrowheads). The patient
was a 37-year-old woman with sarcoidosis.

■ FIGURE 7-14 Irregular septal thickening in


idiopathic pulmonary fibrosis. High-resolution CT scan
shows a few bilateral irregularly thickened septa
(arrows). Note also presence of smaller intralobular
lines resulting in a fine reticular pattern, architectural
distortion, and subpleural distribution of findings
characteristic of idiopathic pulmonary fibrosis. The
patient was a 70-year-old woman.

pulmonary fibrosis, asbestosis, and sarcoidosis (Fig. typically consist of predominantly peripheral and basal
7-14).23,24 It is usually associated with other findings of reticulation and, commonly, honeycombing. The main
fibrosis, such as reticulation, traction bronchiectasis, and parenchymal abnormality in sarcoidosis is nodular thick-
bronchiolectasis, and is seldom the predominant pattern. ening along the bronchi and vessels, typically most severe
The main abnormalities in idiopathic pulmonary fibrosis in the perihilar regions and upper lobes.

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166 PART TWO ● Radiologic Manifestations of Lung Disease

KEY POINTS: DIFFERENTIAL


DIAGNOSIS OF SEPTAL PATTERN
■ Hydrostatic pulmonary edema—smooth; mainly in depen-
dent lung regions
■ Lymphangitic carcinomatosis—usually smooth; some-
times nodular
■ Lymphoma and leukemia—smooth or nodular
■ Churg-Strauss syndrome—smooth; bilateral; symmetric
or asymmetric
■ Congenital lymphangiectasia—smooth; bilateral; associ-
ated with pleural changes
■ Niemann-Pick syndrome—smooth or nodular; bilateral
■ Erdheim-Chester disease—smooth; diffuse; associated
with pleural changes
■ Yellow nail syndrome—smooth; mild
■ Kaposi sarcoma—smooth or nodular
■ Pulmonary hemorrhage—smooth; usually associated with
ground-glass opacities
■ Lymphoid interstitial pneumonia—smooth; associated
with ground-glass opacities or centrilobular nodules or
both
■ Amyloidosis—rare; smooth or nodular
■ Acute respiratory distress syndrome (ARDS)—smooth;
usually associated with ground-glass opacities (“crazy
paving” pattern)
■ Idiopathic pulmonary fibrosis—irregular; associated with
reticulation
■ Asbestosis—irregular; associated with reticulation
■ Sarcoidosis—nodular or irregular; seldom the predomi-
nant pattern
■ Silicosis—nodular; rare
■ Coal workers’ pneumoconiosis—nodular; rare
■ Others ■ FIGURE 7-15 Schematic drawing of reticular pattern. Schematic
● Acute lung rejection—smooth drawing of the right lung illustrates reticular pattern. A reticular pattern
● Pleural inflammation—smooth; unilateral on high-resolution CT typically results from intralobular lines (i.e., fine
● Idiopathic bronchiectasis—smooth; central or linear opacities visible within a pulmonary lobule as a result of
peripheral thickening of the intralobular interstitial tissue).

RETICULAR PATTERN
nia.28 The radiographic findings consist of a symmetric,
A reticular pattern is characterized by innumerable, inter- bilateral reticular pattern that may be diffuse, but tends to
lacing line shadows that suggest a mesh (Fig. 7-15).25 On involve predominantly the lower lung zones (see Fig.
the chest radiograph, the pattern may be the result of 7-16).29,30 In 60% of patients, a predominant peripheral
summation of smooth or irregular linear opacities, cystic distribution is apparent on the radiograph.22 As the disease
spaces, or both (Fig. 7-16). Although distinction between progresses, the reticular pattern becomes coarser and is
these abnormalities often is difficult on the radiograph, it associated with progressive loss of volume.
can be made readily on high-resolution CT. The reticular The characteristic high-resolution CT findings of idio-
pattern on high-resolution CT results from small irregular pathic pulmonary fibrosis consist of a reticular pattern in
intralobular linear opacities separated by only a few milli- a patchy distribution and involving all lobes, but being
meters (Figs. 7-17 and 7-18). Intralobular linear opacities most severe in the subpleural lung regions and in the lung
reflect thickening of the interstitium within the secondary bases (Fig. 7-19).31,32 The reticular pattern is usually associ-
pulmonary lobule and are most commonly caused by ated with irregular pleural, vascular, and bronchial inter-
fibrosis. Fibrosis also typically results in distortion of the faces; evidence of architectural distortion; and dilation
parenchymal architecture, traction bronchiectasis, and of bronchi and bronchioles (traction bronchiectasis
traction bronchiolectasis (see Fig. 7-18).26,27 Common and bronchiolectasis) (see Figs. 7-18 and Fig. 7-19).26,33
causes of a reticular pattern include idiopathic interstitial Air-containing cysts measuring 2-20 mm in diameter (hon-
fibrosis, nonspecific interstitial pneumonia, fibrosis associ- eycombing) are present in 80%-90% of patients at
ated with collagen vascular disease, chronic hypersensitiv- diagnosis.34,35
ity pneumonitis, sarcoidosis, and asbestosis. A reticular pattern also frequently is seen in patients
Idiopathic pulmonary fibrosis is an idiopathic chronic with nonspecific interstitial pneumonia.31 Nonspecific
interstitial fibrosis limited to the lung and associated with interstitial pneumonia is a chronic interstitial lung dis-
the histologic appearance of usual interstitial pneumo- ease characterized histologically by a combination of

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CHAPTER 7 ● Interstitial Patterns 167

B
■ FIGURE 7-16 Reticular pattern on the chest radiograph.
A, Posteroanterior chest radiograph shows extensive bilateral reticular
pattern worse in the lower lung zones. B, Magnified view of the left lower
lung zone shows more clearly the reticular pattern (arrows). The patient
was a 58-year-old man with idiopathic pulmonary fibrosis. The reticular
pattern in this patient was caused by summation of irregular linear
opacities and honeycombing.

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168 PART TWO ● Radiologic Manifestations of Lung Disease

B
■ FIGURE 7-17 Reticular pattern on high-resolution CT. A and B, High-resolution CT
scans at the level of aortic arch (A) and bronchus intermedius (B) show bilateral
subpleural irregular intralobular linear opacities and irregular septal thickening resulting
in a fine reticular pattern. The patient was a 73-year-old woman with idiopathic
pulmonary fibrosis.

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CHAPTER 7 ● Interstitial Patterns 169

B
■ FIGURE 7-18 Severe reticular pattern with traction bronchiectasis and
bronchiolectasis. A, High-resolution CT scan at the level of the lung bases shows
reticular pattern in the peripheral regions of the lower lobes and diffusely in the
middle lobe and lingula. B, Magnified view of the left lower lung zone shows
reticular pattern. Also noted is dilation and beading of bronchi (traction
bronchiectasis) (straight arrows). Dilated airways within approximately 0.5-1 cm
from the pleura represent ectatic bronchioles (traction bronchiolectasis) (curved
arrows). The patient was a 70-year-old man with idiopathic pulmonary fibrosis.

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170 PART TWO ● Radiologic Manifestations of Lung Disease

A B

■ FIGURE 7-19 Idiopathic pulmonary fibrosis. A, High-resolution CT


scan at the level of the bronchus intermedius shows bilateral reticular
pattern involving almost exclusively the peripheral lung regions. B, High-
resolution CT scan at the level of the lung bases shows more extensive
reticulation and subpleural honeycombing. C, Coronal reformation shows
reticulation in all lobes, but most severe in the subpleural lung regions
and lung bases. The patient was a 70-year-old man with idiopathic
pulmonary fibrosis.
C

interstitial fibrosis and inflammation that resembles idio- ing, and relative sparing of the subpleural regions allow
pathic pulmonary fibrosis clinically, but has a considerably distinction of nonspecific interstitial pneumonia from idio-
better prognosis. Nonspecific interstitial pneumonia may pathic pulmonary fibrosis in most cases.36,39 It may be
be idiopathic, but is seen most commonly in patients with impossible, however, to distinguish fibrotic nonspecific
connective tissue disease, particularly scleroderma, and interstitial pneumonia with a predominantly reticular
drug reaction. The radiographic and high-resolution CT pattern from idiopathic pulmonary fibrosis.
findings most commonly consist of ground-glass opacities The most common collagen vascular diseases associ-
and a fine reticular pattern (Fig. 7-20).36,37 Evidence of ated with a reticular pattern on the chest radiograph and
architectural distortion with traction bronchiectasis and high-resolution CT are scleroderma and rheumatoid arthri-
bronchiolectasis is commonly seen on high-resolution CT, tis. The histologic, radiographic, and high-resolution CT
but honeycombing is uncommon at presentation. The manifestations of scleroderma are usually those of nonspe-
abnormalities may be diffuse, but they involve mainly the cific interstitial pneumonia40,41; rheumatoid arthritis tends
lower lung zones in 60%-90% of cases and predominantly to result in findings of usual interstitial pneumonia and,
the lung periphery in 50%-70%.36,38 Although the fibrosis less commonly, nonspecific interstitial pneumonia.42
in nonspecific interstitial pneumonia is often predomi- In chronic hypersensitivity pneumonitis, the fibrosis is
nantly peripheral and basal, in approximately 50% of situated predominantly in the middle lung zones or shows
patients there is relative sparing of the immediate sub- no zonal predominance (Fig. 7-22).43 Lung apices and
pleural lung in the dorsal regions of the lower lobes (Fig. bases are relatively spared. The distribution of fibrosis on
7-21).39 The presence of predominantly ground-glass opac- high-resolution CT is commonly random, but may be pre-
ities, relatively mild reticulation, absence of honeycomb- dominantly subpleural or peribronchovascular.43 In most

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CHAPTER 7 ● Interstitial Patterns 171

■ FIGURE 7-20 Nonspecific interstitial pneumonia.


A, Posteroanterior chest radiograph shows mild bilateral
reticular pattern and extensive ground-glass opacities mainly in
the middle and lower lung zones. B, High-resolution CT scan
shows bilateral ground-glass opacities with mild superimposed
reticulation in the lower lobes.
Continued

cases, there are superimposed findings of subacute disease, mal abnormalities.17 The fibrosis in sarcoidosis typically
typically poorly defined centrilobular nodular opacities, involves mainly the upper and middle lung zones. It is
extensive bilateral ground-glass opacities, and lobular typically associated with superior retraction of the hila,
areas with decreased attenuation and vascularity with air traction bronchiectasis of the central bronchi, and com-
trapping on expiratory images (see Fig. 7-22).39,44 The pre- pensatory overinflation of the lower lobes (Fig. 7-23).24,45
dominantly midlung zone distribution of fibrosis and the The reticular pattern in asbestosis is usually mild and
presence of centrilobular nodules and lobular areas of air involves predominantly or exclusively the lower lung
trapping allow recognition of hypersensitivity pneumoni- zones. High-resolution CT typically shows subpleural
tis in most cases. In some patients, the findings may mimic reticulation, subpleural curvilinear opacities, and small
those of idiopathic pulmonary fibrosis or nonspecific rounded or branching subpleural opacities involving
interstitial pneumonia.39,44 mainly the dorsal regions of the lower lung zones.46,47 Most
A reticular pattern is seen in 15%-20% of patients who patients have associated pleural plaques or diffuse pleural
have sarcoidosis and radiographically evident parenchy- thickening.46

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172 PART TWO ● Radiologic Manifestations of Lung Disease

■ FIGURE 7-20, cont’d C, Coronal reformation shows that


the abnormalities involve mainly the peripheral lung regions
and lower lung zones. Mild subpleural reticulation is seen in
the right upper lobe (arrows). The patient was a 42-year-old
woman with nonspecific interstitial pneumonia.

■ FIGURE 7-21 Nonspecific interstitial pneumonia: relative


subpleural sparing. High-resolution CT scan in a 60-year-old
man with nonspecific interstitial pneumonia shows extensive
bilateral ground-glass opacities, traction bronchiectasis, and
reticulation. The reticulation is less severe in the lung
immediately adjacent to the pleura (arrows) than in the lung
1 cm away from the pleura (relative subpleural sparing), a
characteristic finding seen in approximately 50% of patients
with fibrotic nonspecific interstitial pneumonia.

KEY POINTS: DIFFERENTIAL DIAGNOSIS OF RETICULAR PATTERN


■ Idiopathic pulmonary fibrosis—typically subpleural and basal ■ Hypersensitivity pneumonitis—midlung zone predominance;
predominance; honeycombing present in most cases; usually with ground-glass opacities, centrilobular nodules,
minimal ground-glass opacities and lobular air trapping
■ Nonspecific interstitial pneumonia—ground-glass opacities ■ Sarcoidosis—upper lobe, perihilar, and peribronchovascular
usually predominate; reticulation typically mild and honey- distribution
combing uncommon at presentation ■ Asbestosis—dorsal regions of lower lung zones; pleural
■ Rheumatoid arthritis—fibrosis usually resembles idiopathic plaques; history of exposure
pulmonary fibrosis
■ Systemic sclerosis—fibrosis usually has pattern of nonspecific
interstitial pneumonia

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CHAPTER 7 ● Interstitial Patterns 173

■ FIGURE 7-22 Chronic hypersensitivity pneumonitis.


A, Posteroanterior chest radiograph shows reticulation in the left middle
and lower lung zones and, to a lesser extent, right middle lung zone
(arrows). B, High-resolution CT scan performed at end inspiration shows
mild subpleural reticulation and honeycombing (arrows), extensive bilateral
ground-glass opacities, and localized areas of decreased attenuation and
vascularity. C, High-resolution CT performed after maximal expiration
shows extensive areas of air trapping (arrows). The patient was a
78-year-old woman with chronic hypersensitivity pneumonitis.
C

CYSTIC PATTERN consist of cysts (present in approximately 80% of patients)


and nodules (present in 60%-80%).51,52 The nodules pre-
Cystic airspaces are round, air-containing parenchymal dominate in the early phases, and the cysts predominate
spaces with a well-defined wall.25 The term is used to in the late stages of the disease.53 The nodules range from
describe enlarged airspaces seen in Langerhans cell histio- 1-10 mm in diameter, and the cysts range from a few mil-
cytosis, lymphangioleiomyomatosis, and lymphoid inter- limeters to several centimeters in diameter and may be
stitial pneumonia; in end-stage fibrosis (honeycombing) round or oval or have bizarre shapes with bilobed, clover-
as may be seen in idiopathic pulmonary fibrosis; and, leaf, and branching configuration.48 Regardless of the stage
less commonly, in chronic hypersensitivity pneumonitis, of the disease, the abnormalities are diffuse throughout
nonspecific interstitial pneumonia, asbestosis, and the middle and upper lung zones with relative sparing of
sarcoidosis. the lung bases (see Fig. 7-24).49
Pulmonary Langerhans cell histiocytosis is a disease Lymphangioleiomyomatosis is a rare disease limited to
characterized histologically by infiltration of the lung by women and characterized by cyst formation and an inter-
Langerhans cells that typically manifests in young adults stitial proliferation of smooth muscle–like cells.54 The
and is seen almost exclusively in smokers.48 The parenchy- radiographic manifestations consist of a diffuse bilateral
mal abnormalities are typically bilateral, symmetric, and reticular pattern and, commonly, evidence of hyperinfla-
diffuse throughout the upper and middle lung zones with tion manifested by an increase in the retrosternal airspace
sparing of the costophrenic angles.49 The chest radiograph or flattening of the diaphragm (Fig. 7-25). Cysts can be
tends to show a reticular or reticulonodular pattern (Fig. identified on the radiograph in 50%-60% of cases.55,56 The
7-24).50 The characteristic findings on high-resolution CT characteristic high-resolution CT finding consists of numer-

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174 PART TWO ● Radiologic Manifestations of Lung Disease

■ FIGURE 7-23 Pulmonary fibrosis in sarcoidosis. A, Chest radiograph


shows extensive bilateral reticulonodular pattern involving mainly the
upper lobes. B, High-resolution CT scan at the level of the upper lobes
shows extensive reticulation with associated distortion of architecture and
traction bronchiectasis (arrows). C, Coronal reformation shows upper lobe
distribution of the reticulation and traction bronchiectasis (arrows). Note
superior retraction of the hila and compensatory overinflation of the lower
lobes. Small perilymphatic nodules in the upper and middle lung zones
typical of sarcoidosis also are evident.
C

ous thin-walled, air-filled cysts surrounded by normal lung few in number and tend to involve mainly the lower lung
parenchyma (see Fig. 7-25).57 The cysts can be seen on zones (Fig. 7-27); occasionally, lymphoid interstitial pneu-
high-resolution CT in patients who have normal radio- monia may result in extensive cyst formation.60 The cysts
graphs or radiographs showing only reticular opacities. in subacute hypersensitivity pneumonitis may be single or
They usually measure 0.2-2 cm in diameter and are distrib- multiple and have a random distribution. In a study of 36
uted diffusely throughout the lungs without any zonal patients with chronic hypersensitivity pneumonitis, Silva
predominance.56,57 and colleagues39 showed the presence of a few cysts in 14
Cystic spaces are seen in approximately 30% of patients patients (39%).39 In most cases, the predominant abnor-
with Pneumocystis jiroveci pneumonia.58 These may be malities of Pneumocystis pneumonia, lymphoid interstitial
single or multiple, occur mainly in the upper lobes, and pneumonia, and subacute hypersensitivity pneumonitis
are typically superimposed on ground-glass opacities (Fig. are ground-glass opacities (see further on).
7-26). The cysts in Pneumocystis pneumonia may repre- Honeycombing refers to the presence of cystic spaces
sent pneumatoceles, premature emphysema, or occasion- usually measuring 0.3-1 cm in diameter whose walls
ally cavitated nodules. consist of a variable amount of fibrous tissue (Fig. 7-28).
Single or multiple cysts typically superimposed on Honeycombing typically tends to be most severe in
ground-glass opacities also are seen in approximately 60% the subpleural regions and along the interlobar fissures
of patients with lymphoid interstitial pneumonia15 and 13% (Fig. 7-29). The most common diseases in which the
of patients with subacute hypersensitivity pneumonitis.59 abnormality is identified are idiopathic pulmonary fibrosis,
The cysts in lymphoid interstitial pneumonia are usually connective tissue disease, and sarcoidosis; however,

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CHAPTER 7 ● Interstitial Patterns 175

A C

B D
■ FIGURE 7-24 Pulmonary Langerhans cell histiocytosis. A, Chest radiograph shows diffuse reticular pattern in the upper and middle lung zones
with sparing of the lower lung zones. B, High-resolution CT scan at the level of the lung apices shows numerous bilateral thin-walled cysts.
Conglomeration of cysts in the left upper lobe has led to the formation of large cysts with bizarre shapes. C, High-resolution CT scan slightly above
the level of the aortic arch shows numerous bilateral cysts, a few small nodules, and ground-glass opacities. D, High-resolution CT scan at the level of
the lung bases shows minimal abnormalities. The patient was a 52-year-old man who was a smoker and developed pulmonary Langerhans cell
histiocytosis. The ground-glass opacities reflect the presence of respiratory bronchiolitis (“smoker’s bronchiolitis”).

the process can be seen in the advanced stage of In most patients with cystic abnormalities, a confident
pulmonary fibrosis of any cause (Fig. 7-30).24 The spaces diagnosis can be made on high-resolution CT based on the
represent mainly respiratory bronchioles and alveolar appearance and distribution of the cystic changes and the
ducts that have become dilated as a result of traction presence of associated findings, such as nodules and
by the adjacent fibrous tissue.61 The presence, distribu- ground-glass opacities (Fig. 7-31).62,63 Similar to other inter-
tion, and extent of honeycombing are assessed much stitial findings, the final diagnosis requires integration of
more readily on high-resolution CT scan than on chest the high-resolution CT findings with the clinical informa-
radiograph. The differential diagnosis of conditions associ- tion and laboratory data. Some patients may require lung
ated with honeycombing is similar to that of biopsy for a definitive diagnosis.
reticulation. Text continued on p. 181.

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B

■ FIGURE 7-25 Lymphangioleiomyomatosis. A, Posteroanterior chest


radiograph shows diffuse fine reticular pattern with distortion of the
pulmonary vasculature and small areas of lucency. B, High-resolution CT
scan at the level of the upper lobes shows numerous bilateral thin-walled
cysts. C, High-resolution CT scan at the level of the lung bases shows
diffuse involvement similar to that in the upper lobes. D, Coronal
reformation shows diffuse distribution of cysts and large lung volumes.
E, Sagittal reformation of the right lung shows diffuse distribution of the
cysts and increase in the anteroposterior diameter of the chest owing to
increased lung volume. The patient was a 75-year-old woman with
long-standing lymphangioleiomyomatosis.
D

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CHAPTER 7 ● Interstitial Patterns 177

■ FIGURE 7-26 Pneumocystis pneumonia. High-resolution CT scan at the level of the


upper lobes shows numerous bilateral cysts of various sizes and evidence of emphysema.
Ground-glass opacities adjacent to the cystic spaces also are noted. Paraseptal and
centrilobular emphysema also is present. The patient was a 35-year-old man with AIDS
and Pneumocystis jiroveci pneumonia.

■ FIGURE 7-27 Lymphoid interstitial pneumonia. High-resolution CT scan shows


patchy bilateral ground-glass opacities and several thin-walled cysts (arrows). The patient
was a 44-year-old woman with lymphoid interstitial pneumonia who was a lifelong
nonsmoker.

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178 PART TWO ● Radiologic Manifestations of Lung Disease

■ FIGURE 7-29 Honeycombing: histologic findings. Histologic


specimen in a patient with idiopathic pulmonary fibrosis shows
characteristic peripheral predominance of fibrosis with associated
architectural distortion and cystic spaces with well-defined walls.

■ FIGURE 7-28 Schematic drawing of honeycombing. Schematic


drawing of the right lung illustrates honeycombing. Honeycombing is
characterized on high-resolution CT scan by the presence of clustered
cystic airspaces with well-defined walls and typically measuring 3-10 mm
in diameter. Honeycombing is a CT feature of established pulmonary
fibrosis.

A B
■ FIGURE 7-30 Honeycombing in end-stage idiopathic pulmonary fibrosis. A, Posteroanterior chest radiograph shows bilateral reticular pattern
mainly in the peripheral lung and lower lung zones. B, High-resolution CT scan obtained on a multidetector scanner shows extensive subpleural
honeycombing.

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CHAPTER 7 ● Interstitial Patterns 179

■ FIGURE 7-30, cont’d C, Coronal reformation image shows


peripheral and basal distribution of the honeycomb cysts. The patient was
a 59-year-old man with idiopathic pulmonary fibrosis.

A B
■ FIGURE 7-31 Schematic drawing of cystic pattern and distribution. A and B, Pulmonary Langerhans histiocytosis is characterized by the presence
of nodules and cysts (A) or, in the late stage, only cysts in the middle and upper lung zones with relative sparing of the lung bases (B).
Continued

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180 PART TWO ● Radiologic Manifestations of Lung Disease

C D

F
E
■ FIGURE 7-31, cont’d C, The cysts in lymphangiomyomatosis have a random distribution throughout both lungs. D, Honeycombing typically
involves mainly the peripheral and basal lung regions. E, The cysts in Pneumocystis pneumonia typically are superimposed on ground-glass opacities
and have upper lobe predominance. F, The cysts in lymphocytic interstitial pneumonia and subacute hypersensitivity pneumonitis have a random
distribution, but usually occur in areas with ground-glass opacity and tend to involve mainly the middle and lower lung zones.

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CHAPTER 7 ● Interstitial Patterns 181

ening of the bronchovascular bundles. Another sign


KEY POINTS: DIFFERENTIAL helpful in assessing the perilymphatic distribution is the
DIAGNOSIS OF CYSTIC PATTERN presence of subpleural nodules in relation to the interlo-
bar fissures, a characteristic finding in sarcoidosis, silico-
■ Langerhans cell histiocytosis—cysts and nodules diffuse in sis, and coal workers’ pneumoconiosis (see Fig. 7-32). The
the upper and middle lung zones; relative sparing of lung various diseases associated with a perilymphatic distri-
bases bution of nodules usually can be distinguished on high-
■ Lymphangioleiomyomatosis—cysts diffuse throughout
resolution CT by the different patterns of involvement of
the lungs; seen only in women, typically childbearing
age the perilymphatic interstitium and the presence of associ-
■ Pneumocystis pneumonia—present in 30% of cases; single ated findings.
or multiple; superimposed on ground-glass opacities; The most common radiologic manifestation of sarcoid-
usually in upper lobes osis is symmetric bilateral hilar and mediastinal lymph
■ Lymphoid interstitial pneumonia—present in 60% of node enlargement with or without associated parenchy-
cases; superimposed on ground-glass opacities; frequently mal abnormalities. Parenchymal involvement typically
multiple; mainly in lower lobes results in a nodular or reticulonodular pattern involving
■ Hypersensitivity pneumonitis—present in 13%-39% of mainly the upper and middle lung zones (Fig. 7-34).17,22
cases; superimposed on ground-glass opacities; random On high-resolution CT, nodules are seen at initial evalua-
distribution
tion in 90%-100% of patients who have parenchymal
■ Idiopathic pulmonary fibrosis—peripheral and basal pre-
dominance; cystic pattern owing to honeycombing abnormalities.45,65 The nodules are typically most numer-
ous along the bronchoarterial and pleural interstitium and
adjacent to the interlobar fissures (Fig. 7-35). This distribu-
tion of nodules is highly suggestive of sarcoidosis. Although
nodular septal thickening is often evident on high-
NODULAR PATTERN resolution CT, this seldom is the predominant finding.
In most patients with lymphangitic spread of tumor,
A nodular pattern is characterized by the presence of the main finding is interlobular septal thickening (see
numerous round opacities measuring less than 1 cm in Fig. 7-2). The septal thickening may be smooth or, less
diameter. The pattern results from expansion of the paren- commonly, nodular. Peribronchovascular and subpleural
chymal interstitium by a roughly spherical cellular infil- nodules may be seen, but they are typically not as profuse
trate, fibrous tissue, or both. The differential diagnosis as in patients with sarcoidosis.66 Other common findings
is based on the pattern and distribution of the nodules, seen in patients with lymphatic spread of tumor are uni-
the presence of associated findings such as lymph node lateral or asymmetric hilar lymph node enlargement and
enlargement, and the clinical setting. A diffuse small pleural effusion.
nodular pattern in a febrile patient with acute disease is Silicosis and coal workers’ pneumoconiosis are associ-
most suggestive of hematogenous infection, particularly ated with the presence of small, well-defined nodules,
miliary tuberculosis. A similar pattern in a patient with usually measuring 2-5 mm in diameter. Similar to sarcoid-
chronic symptoms may result from silicosis, coal workers’ osis, silicosis and coal workers’ pneumoconiosis can be
pneumoconiosis, intravenous talcosis, metastatic carci- diffuse, but usually involve mainly the middle and upper
noma (particularly from the thyroid), and bronchioloalve- long zones and frequently are associated with hilar and
olar carcinoma. The overall distribution of nodules on the mediastinal lymph node enlargement. Similar to sarcoid-
radiograph often helps to narrow the differential diagno- osis, nodules are frequently seen in the subpleural regions
sis. The nodules in silicosis and coal workers’ pneumoco- and along the interlobar fissures. The nodules in silicosis
niosis may be diffuse, but most commonly involve mainly and coal workers’ pneumoconiosis are predominantly
the middle and upper lung zones, whereas the nodules centrilobular in distribution, however, and typically are
resulting from hematogenous processes, such as miliary most numerous in the dorsal half of the upper lobes (Fig.
tuberculosis and metastatic carcinoma, are diffuse or 7-36).18,19 The diagnosis is readily made based on the radio-
involve mainly the lower lung zones (where blood flow is graphic or CT findings and exposure history.
greater).29,64 The various diseases resulting in multiple
nodules typically result in three main patterns of distribu-
tion on high-resolution CT: perilymphatic, centrilobular,
Centrilobular Nodular Pattern
and random (Fig. 7-32). Centrilobular nodules are characterized on high-resolution
CT by their location several millimeters away from the
Perilymphatic Nodular Pattern pleural surfaces, interlobar fissures, and interlobular septa.
They reflect the presence of bronchiolocentric interstitial
A perilymphatic distribution along the bronchovascular lung disease or bronchiolitis. Centrilobular nodules are
interstitium, interlobular septa, and subpleural lung typically seen in hypersensitivity pneumonitis39,44; various
regions is characteristically seen in patients with sarcoid- forms of bronchiolitis including respiratory bronchiol-
osis (Fig. 7-33),65 lymphangitic carcinomatosis,7 silicosis, itis,67 respiratory bronchiolitis–interstitial lung disease
and coal workers’ pneumoconiosis.18,19 In these condi- (RB-ILD),67 Asian panbronchiolitis,68 and infectious bron-
tions, the nodules tend to be well defined and usually chiolitis69; and endobronchial spread of tuberculosis or
measure 2-5 mm in diameter. Nodules in a perilymphatic Mycobacterium avium-intracellulare infection.70 Centri-
distribution frequently are associated with nodular thick- lobular nodules in hypersensitivity pneumonitis, respira-

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182 PART TWO ● Radiologic Manifestations of Lung Disease

A B
■ FIGURE 7-32 Schematic drawing of nodular pattern and distribution. A, Perilymphatic distribution is characterized by the presence of nodules
along the bronchovascular interstitium, interlobular septa, and subpleural lung regions. B and C, Centrilobular distribution is characterized by the
presence of nodules a few millimeters away from the interlobular septa, visceral pleura, interlobar fissures, large vessels, and bronchi. Centrilobular
nodules that are well defined have a tree-in-bud appearance and in a patchy distribution (B) are seen most commonly in infectious bronchiolitis.

tory bronchiolitis, and RB-ILD typically have poorly defined with mucus, fluid, or pus, and often associated with
margins, whereas nodules associated with infectious peribronchiolar inflammation. It usually is associated
bronchiolitis and endobronchial spread of tuberculosis with infection of the small airways, the most common
usually have well-defined margins and are commonly as- causes being infectious bronchiolitis, bronchopneumonia,
sociated with branching opacities resulting in a tree-in- and endobronchial spread of tuberculosis or M. avium-
bud appearance. intracellulare (Figs. 7-40 and 7-41).69,72 The tree-in-bud
In hypersensitivity pneumonitis, the nodules are usually pattern also can be seen distal to bronchiectasis of any
diffuse throughout the lungs or involve mainly the middle cause72 and in allergic bronchopulmonary aspergillosis.73
and lower lung zones and are frequently associated with The various forms of infectious bronchiolitis and endo-
ground-glass opacities and lobular areas of decreased bronchial spread of mycobacterial infection tend to result
attenuation and vascularity with air trapping on expiratory in well-defined centrilobular nodules and branching opaci-
CT (Figs. 7-37 and 7-38).44 The centrilobular nodules in ties (tree-in-bud pattern) in a focal or multifocal unilateral
respiratory bronchiolitis and RB-ILD tend to involve pre- or bilateral distribution (see Fig. 7-41). They usually can
dominantly or exclusively the upper lobes, and because be readily distinguished from diffuse interstitial processes
these conditions are seen exclusively in cigarette smokers, such as hypersensitivity pneumonitis and RB-ILD that typi-
they are frequently associated with centrilobular emphy- cally result in centrilobular nodular opacities that are
sema (Fig. 7-39).67,71 poorly defined (ground-glass), have a bilateral and sym-
metric distribution, and are seldom associated with a tree-
in-bud pattern. Occasionally, centrilobular nodules and a
Tree-in-Bud Pattern tree-in-bud pattern may result from abnormalities related
to the centrilobular pulmonary arteries, such as may occur
The tree-in-bud pattern usually reflects the presence of in intravascular metastases (Fig. 7-42).74
dilated centrilobular bronchioles, their lumina impacted Text continued on p. 189.

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CHAPTER 7 ● Interstitial Patterns 183

C D
■ FIGURE 7-32, cont’d Poorly defined centrilobular nodular opacities (C) are seen most commonly in bronchiolocentric alveolitis as occurs in
hypersensitivity pneumonitis and respiratory bronchiolitis. D, Random distribution is characterized by a distribution of nodules without any particular
predominance. Some nodules may appear to be centrilobular, whereas others may appear to be perilymphatic.

■ FIGURE 7-33 Perilymphatic distribution


of sarcoid granulomas. Histologic specimen
shows sarcoid granulomas along the pleura,
bronchi, and pulmonary vessels.

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■ FIGURE 7-34 Nodular pattern in sarcoidosis.
Posteroanterior chest radiograph shows numerous
bilateral nodules involving mainly the upper and
middle lung zones. Also note evidence of right
paratracheal lymph node enlargement (arrow). The
patient was a 37-year-old woman with sarcoidosis.

A B

■ FIGURE 7-35 Perilymphatic nodules in sarcoidosis. A, High-


resolution CT scan at the level of the middle lobe bronchus shows
numerous small nodules located mainly along the bronchi (broad arrows),
vessels (narrow arrow), and interlobar fissures (arrowheads). B, Maximum
intensity projection image shows peribronchial (broad straight arrows) and
perivascular (narrow straight arrow) predominance of nodules. Also note
presence of subpleural nodules and nodules along interlobular septa
(curved arrow) and interlobar fissures (arrowheads). C, Coronal maximum
intensity projection image shows that the nodules involve mainly the
upper lobes and are distributed predominantly along bronchi (broad
straight arrows), vessels (narrow straight arrow), subpleural regions (curved
arrow), and interlobar fissures (arrowheads). The patient was a 28-year-old
C man with sarcoidosis.
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CHAPTER 7 ● Interstitial Patterns 185

C
■ FIGURE 7-36 Silicosis. A, View of the right upper lobe from a posteroanterior chest radiograph shows small nodules. B, High-resolution CT scan
shows small well-defined nodules mainly in the dorsal half of the upper lobes. Some of the nodules can be seen to be in a subpleural (straight arrows)
and centrilobular (arrowheads) distribution. C, Coronal maximum intensity projection image better shows the nodules and their predominantly
centrilobular (curved arrows) distribution. Also seen are a few subpleural nodules (straight arrow). D, Sagittal maximum intensity projection image
shows the characteristic predominance of nodules in the dorsal lung regions and the predominant centrilobular distribution (curved arrows).
(Case courtesy of Dr. Ericson Bagatin, Department of Occupational Health, State University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil.)

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186 PART TWO ● Radiologic Manifestations of Lung Disease

■ FIGURE 7-37 Hypersensitivity pneumonitis. Chest radiograph shows


hazy increased opacity (ground-glass opacity) in the middle and lower
lung zones and poorly defined nodular opacities. The patient was a
42-year-old man with hypersensitivity pneumonitis.

■ FIGURE 7-38 Hypersensitivity


pneumonitis. High-resolution CT scan shows
diffuse parenchymal abnormalities consisting
of poorly defined centrilobular nodular
opacities. The centrilobular opacities typically
are a few millimeters away from the pleura
(thick arrows), interlobular septa (arrowheads),
and large vessels (thin arrows) and bronchi.
The patient was a bird breeder with subacute
hypersensitivity pneumonitis.

■ FIGURE 7-39 Respiratory bronchiolitis. High-resolution


CT scan shows poorly defined centrilobular nodules (arrows) in
the upper lobes. The patient was a 33-year-old man with a
20-pack-year smoking history.

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CHAPTER 7 ● Interstitial Patterns 187

■ FIGURE 7-40 Schematic drawing of tree-in-bud


pattern. A tree-in-bud pattern is characterized by
centrilobular branching linear and nodular opacities. It
is most suggestive of infectious bronchiolitis.

■ FIGURE 7-41 Tree-in-bud pattern in infectious


bronchiolitis. A, High-resolution CT scan shows centrilobular
branching nodular and linear opacities resulting in a tree-in-
bud appearance (arrows). B, Coronal maximum intensity
projection image shows tree-in-bud pattern in the lower lobes.
Continued

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188 PART TWO ● Radiologic Manifestations of Lung Disease

■ FIGURE 7-41, cont’d C, Sagittal maximum intensity projection


image of the right lung shows extensive tree-in-bud pattern in the lower
lobe and mild involvement of the middle and right upper lobes. The
patient was a 20-year-old woman with recurrent respiratory infections.

■ FIGURE 7-42 Tree-in-bud pattern as a result of


intravascular metastases. High-resolution CT scan shows
centrilobular nodular and branching opacities (tree-in-bud
pattern) in the periphery of the upper lobe. Also noted is
nodular thickening of the pulmonary vessels. The patient was a
78-year-old man with intravascular metastatic renal cell
carcinoma.

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CHAPTER 7 ● Interstitial Patterns 189

Random Distribution Distinction of perilymphatic, centrilobular, and random


distribution of small nodules is most easily accomplished
Small nodules in a random distribution in relation to struc- by looking first for pleural nodules and nodules arising in
tures of the lung and secondary lobule are seen most relation to the fissures. If subpleural nodules are absent,
commonly in miliary tuberculosis (Fig. 7-43)75 and miliary the pattern is centrilobular. If numerous subpleural or fis-
fungal infections76 and metastases.77 Hematogenous miliary sural nodules are present, the pattern is either perilym-
infections and metastases tend to be most numerous in phatic or random. These two patterns are distinguished
the lung periphery and at the lung bases. Randomly dis- by looking at the distribution of other nodules. If they
tributed nodules can be seen in relation to interlobular are patchy in distribution, and particularly if a distinct
septa, small vessels, and pleural surfaces, but do not have predominance is noted relative to the peribronchovascu-
consistent or predominant distribution to any of these. lar interstitium, interlobular septa, or subpleural regions,
Lung involvement tends to be bilateral and symmetric. the nodules are perilymphatic. If the nodules are distrib-
The random distribution of nodules usually can be recog- uted in a diffuse and uniform manner, the pattern is
nized on high-resolution CT scans, but may be easier to random.
appreciate on maximum intensity projection reconstruc-
tions (Fig. 7-44).

■ FIGURE 7-43 Miliary tuberculosis. A, Chest radiograph


shows numerous 1-2 mm diameter nodules throughout both
lungs (miliary pattern). B, High-resolution CT scan shows
random distribution of numerous nodules. The patient was a
27-year-old woman with miliary tuberculosis.

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190 PART TWO ● Radiologic Manifestations of Lung Disease

■ FIGURE 7-44 Random distribution of nodules in miliary


tuberculosis. A, High-resolution CT scan shows small nodules
in random distribution. B and C, Maximum intensity projection
images in the transverse (B) and coronal (C) planes better
depict the random distribution of the nodules in a patient with
miliary tuberculosis. (Courtesy of Dr. Catherine Beigelman-Aubry,
Pitié-Salpetriere Hospital, Paris.)

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CHAPTER 7 ● Interstitial Patterns 191

KEY POINTS: DIFFERENTIAL


DIAGNOSIS OF SMALL
NODULAR PATTERN
■ Sarcoidosis—peribronchovascular distribution; mainly
upper lobes
■ Hypersensitivity pneumonitis—centrilobular distribution;
frequently with ground-glass opacities and lobular air
trapping
■ Respiratory bronchiolitis—centrilobular; mainly upper
lobes; emphysema commonly present
■ RB-ILD—centrilobular; mainly upper lobes; emphysema is
commonly present; ground-glass opacities commonly
predominate
■ Silicosis—centrilobular and subpleural; mainly upper
lobes
■ Coal workers’ pneumoconiosis—centrilobular and sub-
pleural; mainly upper lobes
■ Endobronchial spread of tuberculosis—centrilobular;
tree-in-bud common; mainly upper lobes
■ Viral, bacterial, fungal bronchiolitis—frequently associ-
ated with tree-in-bud pattern; focal or multifocal; unilat-
eral or bilateral
■ Miliary tuberculosis—random distribution; diffuse
throughout the lungs

GROUND-GLASS PATTERN
A ground-glass pattern is considered to be present when
there is a hazy increase in opacity without obscuration
of underlying vascular markings (Fig. 7-45).25 If the ves-
sels are obscured, the term consolidation is used.78 The ■ FIGURE 7-45 Ground-glass pattern. Maximum intensity projection
abnormality is a frequent and important finding on high- image of the left lung illustrates characteristic appearance of ground-
glass opacity with homogeneous increase in attenuation that does not
resolution CT scan, but often is difficult to recognize on result in obscuration of underlying vessels.
the chest radiograph. On high-resolution CT, ground-glass
opacity reflects the presence of abnormalities below
the resolution limit. Ground-glass opacities may be seen
in many situations, including interstitial disease, airspace The earliest radiographic manifestations consist of a
disease, and increased capillary blood volume resulting granular or hazy opacity (ground-glass pattern) typically
from congestive heart failure or blood flow redistribution. involving mainly the perihilar areas.83,84 As the disease
Although ground-glass opacity is a relatively nonspecific progresses, the hazy ground-glass opacities progress to
finding, the differential diagnosis can be narrowed consid- consolidation, and the pulmonary involvement becomes
erably by assessing the presence of associated findings, diffuse. The characteristic high-resolution CT finding in
such as nodules or reticulation. In patients with associated Pneumocystis pneumonia consists of bilateral ground-
findings such as nodules or reticulation, considering the glass opacities, which may be diffuse or have a distinct
distribution of these findings, rather than the presence of mosaic pattern with areas of normal lung intervening
ground-glass opacities, facilitates the differential diagno- between the ground-glass opacities (see Fig. 7-46).58,80
sis. Clinical information is essential in the assessment of With time, the areas of ground-glass attenuation progress
patients with predominantly or exclusively ground-glass to consolidation. Common ancillary findings include cyst
opacities.79 Bilateral ground-glass opacities as the predomi- formation, seen in approximately 30% of patients, and
nant or only finding in a patient with AIDS are virtually reticulation.58,80
diagnostic of Pneumocystis pneumonia, whereas identical The radiographic manifestations of diffuse pulmonary
findings in a bird breeder are virtually diagnostic of hyper- hemorrhage consist of patchy ground-glass opacities or
sensitivity pneumonitis.80,81 areas of airspace consolidation scattered fairly evenly
Acute lung diseases characteristically associated with a throughout the lungs (Fig. 7-47). The opacities may be
ground-glass pattern include Pneumocystis pneumonia, diffuse or more prominent in the perihilar regions and the
pulmonary hemorrhage, and ARDS (Fig. 7-46). Pneumo- middle and lower lung zones.85 The apices and costo-
cystis pneumonia occurs almost exclusively in immuno- phrenic angles are almost invariably spared.85 The high-
compromised patients, particularly patients with AIDS resolution CT manifestations consist of areas of ground-glass
and patients on long-term immunosuppressive therapy.82 opacity or, less commonly, consolidation; they may be

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192 PART TWO ● Radiologic Manifestations of Lung Disease

■ FIGURE 7-46 Pneumocystis jiroveci pneumonia.


A, Chest radiograph shows symmetric bilateral hazy
increased opacity (ground-glass opacity). B, High-
resolution CT scan at the level of the upper lobes
shows extensive bilateral ground-glass opacities.
C, Coronal reformation shows that the ground-glass
opacities are most extensive in the upper and middle
lung zones. The patient was a 42-year-old woman with
newly diagnosed AIDS and Pneumocystis pneumonia.

C
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CHAPTER 7 ● Interstitial Patterns 193

A B
■ FIGURE 7-47 Diffuse pulmonary hemorrhage. A, Chest radiograph shows ground-glass opacities involving mainly the lower lung zones. B, High-
resolution CT scan shows patchy bilateral ground-glass opacities. The patient was an 18-year-old woman with diffuse pulmonary hemorrhage resulting
from vasculitis.

■ FIGURE 7-48 Hypersensitivity pneumonitis. High-


resolution CT scan shows extensive bilateral ground-glass
opacities, a few centrilobular nodules, and lobular areas of
decreased attenuation and vascularity (arrows). The patient was
a 65-year-old woman with subacute hypersensitivity
pneumonitis.

patchy or diffuse, but tend to involve mainly the depen- nia, RB-ILD, and lymphoid interstitial pneumonia. Hyper-
dent lung regions (see Fig. 7-46).85,86 sensitivity pneumonitis is probably the most common
The earliest radiographic manifestations of ARDS consist cause of diffuse ground-glass opacities in normal hosts.79
of bilateral poorly defined hazy (ground-glass) opacities The areas of ground-glass attenuation in hypersensitivity
and patchy areas of consolidation.87,88 The characteristic pneumonitis may be diffuse, but frequently involve mainly
high-resolution CT findings consist of symmetric bilateral the middle and lower lung zones.44,91 Localized areas of
ground-glass opacities and gravity-dependent consolida- decreased attenuation and perfusion are often present in
tion.87,89 On high-resolution CT, the lung involvement is conjunction with areas of ground-glass attenuation (Fig.
typically heterogeneous with up to one third of the lung 7-48). The areas of low attenuation in hypersensitivity
appearing to be normally aerated.87,90 pneumonitis often have a lobular distribution and show
The differential diagnosis in patients with chronic air trapping on CT scans performed at end expiration.92
respiratory symptoms and exclusively ground-glass opaci- Ground-glass opacities also may be seen in conjunction
ties includes hypersensitivity pneumonitis, nonspecific with centrilobular nodules.44,91 The centrilobular nodules
interstitial pneumonia, desquamative interstitial pneumo- are typically poorly defined, measure less than 5 mm in

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194 PART TWO ● Radiologic Manifestations of Lung Disease

diameter, and are most numerous in the middle and lower lower lung zones.96 A fine reticular pattern in a predomi-
lung zones. Approximately 13% of patients with subacute nantly subpleural and basal distribution is seen in 60%-80%
hypersensitivity pneumonitis have thin-walled lung cysts of patients.96,97
ranging from 3-25 mm in maximal diameter and 1-15 in Lymphoid interstitial pneumonia is an uncommon
number in a random distribution.59 benign lymphoproliferative disorder seen mainly in
Nonspecific interstitial pneumonia is a chronic intersti- patients with underlying immunologic disorders, most
tial lung disease characterized histologically by varying commonly Sjögren’s syndrome. The radiographic manifes-
degrees of alveolar wall inflammation and fibrosis.93 Non- tations usually consist of a reticular or reticulonodular
specific interstitial pneumonia may be idiopathic or be a pattern involving mainly the lower lung zones.98 The most
manifestation of collagen vascular disease, drug reaction, common abnormalities on high-resolution CT consist of
or hypersensitivity pneumonitis.93 It may precede the bilateral areas of ground-glass attenuation and poorly
diagnosis of collagen vascular disease by several months defined centrilobular nodules; approximately 60% of
or several years.93 The most common high-resolution patients have one or more cystic airspaces (see Fig.
CT manifestation consists of bilateral symmetric ground- 7-27).15
glass opacities.36,37 Most patients have a fine reticular
pattern superimposed on the ground-glass opacities, “Crazy Paving” Pattern
traction bronchiectasis, and architectural distortion.36,37
The abnormalities in nonspecific interstitial pneumonia The “crazy paving” pattern consists of patchy or diffuse
tend to involve mainly the lower lung zones; a periph- ground-glass opacities with superimposed interlobular
eral predominance is seen in 50%-70% of patients septal thickening and intralobular lines.99,100 This pattern
(Fig. 7-49).36,37 was first described in patients with pulmonary alveolar
Patients who have RB-ILD are typically current smokers proteinosis (Fig. 7-50)100 and is typical of pulmonary alveo-
with more than 30 pack-years of cigarette smoking.93 The lar proteinosis, but also may be seen in patients with a
chest radiograph is often normal or shows nonspecific variety of other diseases, including ARDS (Fig. 7-51),99
findings related to chronic obstructive pulmonary disease. acute interstitial pneumonia,99 pulmonary edema,6 pulmo-
The high-resolution CT manifestations of RB-ILD typically nary hemorrhage,85 bacterial pneumonia,99 Pneumocystis
consist of ground-glass opacities and poorly defined cen- pneumonia,101 acute eosinophilic pneumonia,102 Churg-
trilobular nodules.67,94 The abnormalities can be diffuse or Strauss syndrome,103 radiation pneumonitis,104 bronchio-
involve mainly the upper or lower lung zones. Most loalveolar carcinoma,105 and lipoid pneumonia.106 The
patients have associated centrilobular emphysema. differential diagnosis is broad and similar to the differential
Desquamative interstitial pneumonia is an uncommon diagnosis of ground-glass opacities is based largely on the
condition, characterized histologically by the presence of presence and distribution of associated findings, such as
numerous macrophages filling the alveolar airspaces, mild consolidation and knowledge of whether symptoms are
inflammation of the alveolar walls, and mild fibrosis.93 acute or chronic.
Approximately 90% of patients who have desquamative
interstitial pneumonia are current cigarette smokers.95
The most common radiographic and high-resolution CT
finding consists of symmetric bilateral ground-glass opaci-
ties.95,96 The ground-glass opacities may be patchy or KEY POINTS: DIFFERENTIAL
diffuse, but in approximately 60% involve mainly the DIAGNOSIS OF GROUND-GLASS
PATTERN
■ Pneumocystis pneumonia—acute; immunocompromised
patient
■ Cytomegalovirus pneumonia—acute; immunocompro-
mised patient
■ Pulmonary hemorrhage—acute; usually present with
hemoptysis
■ ARDS—acute; dependent consolidation commonly
present
■ Hypersensitivity pneumonitis—acute, subacute or chronic;
commonly have centrilobular nodules and lobular areas
of air trapping
■ Nonspecific interstitial pneumonia—chronic; commonly
with fine reticulation
■ RB-ILD—chronic; commonly with centrilobular nodules;
smokers
■ Desquamative interstitial pneumonia—rare; history of
cigarette smoking
■ Lymphoid interstitial pneumonia—rare; usually have
■ FIGURE 7-49 Nonspecific interstitial pneumonia. High-resolution
underlying disease most commonly Sjögren’s syndrome
CT scan at the level of the lung bases shows extensive bilateral ground- ■ Alveolar proteinosis—chronic; rare; often with septal
glass opacities. The patient was a 62-year-old man with nonspecific thickening (“crazy paving”)
interstitial pneumonia.

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CHAPTER 7 ● Interstitial Patterns 195

■ FIGURE 7-50 “Crazy paving” pattern in alveolar


proteinosis. A, Chest radiograph shows extensive bilateral
ground-glass opacities and areas of consolidation with relative
sparing of the lung apices and bases. B, High-resolution CT
scan shows bilateral ground-glass opacities with superimposed
smooth septal lines and intralobular lines resulting in a pattern
known as “crazy paving.” The patient was a 45-year-old man
with alveolar proteinosis.

■ FIGURE 7-51 “Crazy paving” pattern in acute respiratory distress


syndrome. High-resolution CT scan shows bilateral ground-glass opacities
and areas of consolidation involving mainly the dependent lung regions.
Smooth septal lines and intralobular lines are present in the areas of
ground-glass opacity (“crazy paving” pattern). The patient was a
70-year-old man with acute respiratory distress syndrome.

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196 PART TWO ● Radiologic Manifestations of Lung Disease

KEY POINTS: MOST COMMON DIFFERENTIAL DIAGNOSIS OF


“CRAZY PAVING” PATTERN
■ Pulmonary alveolar proteinosis ■ Pneumocystis pneumonia
■ ARDS ■ Churg-Strauss syndrome
■ Acute interstitial pneumonia ■ Radiation pneumonitis
■ Pulmonary edema ■ Bronchioloalveolar carcinoma
■ Pulmonary hemorrhage ■ Lipoid pneumonia
■ Bacterial pneumonia

LIMITATIONS OF THE PATTERN APPROACH times produced by the diffuse interstitial disease itself. On
the radiograph, airspace consolidation superimposed on
The pattern of parenchymal abnormality seen on the chest emphysema may simulate interstitial lung disease, whereas
radiograph and high-resolution CT scan may be modified conglomerate interstitial fibrosis may simulate airspace
by associated underlying parenchymal lung disease, par- disease (Fig. 7-52).
ticularly emphysema, and by the secondary effects some-

A B
■ FIGURE 7-52 Consolidation superimposed on emphysema mimicking interstitial lung disease. A, Magnified view of the left lower lung zone from
a chest radiograph shows hazy increased opacity and reticular pattern. The appearance suggests interstitial lung disease. B, High-resolution CT scan
shows bilateral areas of consolidation and extensive emphysema. The reticular pattern on the radiograph results from consolidation superimposed on
emphysema, rather than interstitial lung disease. The patient was a 70-year-old man with methicillin-resistant Staphylococcus aureus pneumonia and
emphysema.

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