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2ESIDENTS 3ECTION s 0AT TERN OF THE -ONTH

Boitsios et al.
Diffuse Pulmonary Nodules

Residents’ Section
Pattern of the Month
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Residents

inRadiology Diffuse Pulmonary Nodules


Grammatina Boitsios1

A
diffuse pattern of pulmonary nodules, ranging from a few millimeters to 1 cm in
Alexander A. Bankier diameter, may indicate interstitial or airspace disease (Algorithm 1). The pre-
Ronald L. Eisenberg dominant location of the nodules may provide a clue to the underlying condition.
For example, fissural or pleural surfaces are frequently involved in sarcoidosis.
Boitsios G, Bankier AA, Eisenberg RL Upper lobe predominance suggests silicosis or coal workers’ pneumoconiosis; whereas lower
lobe preponderance is more likely in asbestosis or hematogenous metastases, and bronchocen-
tric predominance suggests sarcoidosis or Kaposi sarcoma. The nodules may have a miliary
distribution (tuberculosis, silicosis, sarcoidosis, metastases), cavitate (septic emboli, metasta-
ses, infection) (Fig. 1), or calcify (tuberculosis, silicosis, metastases).
Multiple lung nodules may have a random or centrilobular pattern. Random nodules can
have variable density and are most often uniformly distributed (Fig. 2). The nodules are
typically of hematogenous origin and thus are situated close to small vessels. They may also
occur near the pleural surfaces. In silicosis, the random nodules predominate in the middle
and upper lung zones, whereas metastases are most prevalent in the lung bases.
Centrilobular nodules are not adjacent to the pleural surfaces and the interlobular septa, often
separated from them by a lucent rim. Centrilobular nodules are usually caused by diseases in
which the original lesion develops near bronchioles. Nodules may presents with a ground-glass
appearance when there is involvement of the adjacent peribronchiolar airspaces (Fig. 3).
The tree-in-bud pattern is a manifestation of inflammatory, usually infectious, disease af-
fecting the smallest bronchi and their adjacent alveoli. The “trees” represent dilated bronchi-
oles filled with mucus, pus, or fluid; the “buds” are due to clusters of filled alveoli that have
poorly defined margins and are seen in a centrilobular location.

Diffuse pulmonary nodules

Fissural/pleural surfaces

Keywords: diffuse pattern, pulmonary nodules


Yes No
DOI:10.2214/AJR.10.4345

Received January 22, 2010; accepted after revision


Perihilar versus Random Centrilobular
February 16, 2010.

1
All authors: Department of Radiology, Beth Israel
Deaconess Medical Center and Harvard Medical School, Clustered? Tree-in-bud?
330 Brookline Ave., Boston, MA 02115. Address
correspondence to R. L. Eisenberg (rleisenb@bidmc.
harvard.edu).
Yes No Yes No
WEB
This is a Web exclusive article.

AJR 2010; 194:W354–W366 Sarcoidosis Malignant Small airway Hypersensitivity


Infection
Silicosis entities disease pneumonitis
0361–803X/10/1945–W354

© American Roentgen Ray Society Algorithm 1—Flow chart shows algorithm for treatment of multinodular disease.

W354 AJR:194, May 2010


Diffuse Pulmonary Nodules
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A B
Fig. 1—Cavitation in septic emboli.
A and B, CT images at level of carina (A) and lung bases (B) show emboli as primarily peripheral nodules (white
arrows), one of which is cavitated (black arrow).

Sarcoidosis
Sarcoidosis is a systemic inflammatory
disease that predominantly involves the
lungs but also may affect the joints, eyes,
kidneys, and skin. The disease most com-
monly develops between the ages of 25 and
40 years. Although the exact cause of sarcoi-
dosis is unknown, it may reflect a dispropor-
tional immunologic reaction against a bacte-
rial or environmental antigen. The diagnosis
of sarcoidosis is often made when character-
istic findings are detected incidentally on a
Fig. 2—Random nodules in metastatic melanoma.
chest radiograph obtained for unrelated CT image at level of lung bases shows metastases
symptoms. Patients may have persistent of varying sizes and shapes throughout lung
cough or such systemic manifestations as parenchyma (arrows).
erythema nodosum, uveitis, arthralgia, ar-
thritis, and hypercalcemia. The most common complication of sarcoidosis is respiratory fail-
ure due to pulmonary fibrosis. Cardiac involvement can result in myocardial infarction, ar-
rhythmias, or even sudden death.

A B
Fig. 3—Centrilobular nodules in aspiration pneumonia. CT at level of right lower lobe in patient with history of
chronic aspiration.
A and B, CT image obtained at level of right lower lobe vein (A) shows scattered ground-glass nodules (arrows).
At slightly more caudal level (B), these nodules coalesce into larger areas of ground-glass opacities (arrows).

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Boitsios et al.

Fig. 4—Sarcoidosis.
A, CT image through lung base shows typical interstitial distribution of
small but well-defined nodules (arrow).
B, On different image, there is subpleural mass (black arrow). However,
interstitial nodules are still clearly visible at periphery (white arrow).
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A B

The classic radiographic appearance of bilaterally symmetric enlargement of hilar and para-
tracheal nodes develops in up to 90% of patients with sarcoidosis. The outer borders of the en-
larged hila are usually lobulated. About 20% of patients have diffuse parenchymal disease,
most commonly a reticulonodular pattern that predominantly involves the upper lung zones and
may lead to extensive interstitial pulmonary fibrosis. The nodal enlargement typically resolves
as the parenchymal disease develops, unlike lymphoma or tuberculosis. The bilateral symmetry
differs from tuberculosis, and there is no retrosternal involvement as in lymphoma.
On thin-section CT, sarcoidosis typically presents as irregular micronodules (1–5 mm)
with interstitial thickening that extends along the bronchovascular bundles from the hilum to
the periphery, predominantly in the upper lung zones (Figs. 4 and 5). In late stages, fibrous
distortion of lung parenchyma may be seen at CT before it is apparent on chest radiographs.
Other manifestations of sarcoidosis at CT include ground-glass opacities, alveolar sarcoidosis
(airspace nodules and consolidation with air bronchograms), cysts and cavitation (in necro-
tizing sarcoidal angiitis), and bronchial wall thickening and airway stenoses.

A B

Fig. 5—Sarcoidosis.
A–C, CT images at various anatomic levels show
primarily perihilar interstitial nodules (arrows) in
typical distribution.
C

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Diffuse Pulmonary Nodules

Fig. 6—Silicosis.
Numerous lung
nodules bilaterally
with relative sparing
of lung periphery. Note
subpleural nodules
(arrows). Nodules are
smoothly marginated
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and sharply defined.


(Reprinted with
permission from Webb
WR, Muller NL, Naidich
DP. High-resolution
CT of the lung, 3rd
ed. Philadelphia, PA:
Lippincott Williams &
Wilkins, 2001)

Silicosis
Silicosis is an irreversible occupational lung disease caused by chronic inhalation of dust
containing crystalline silica, which causes diffuse inflammation that leads to lung fibrosis
and emphysema. Silica exposure most often occurs in people who work in construction, min-
ing, sandblasting, stonecutting, and the manufacture of abrasives as well as those who work
with glass, pottery, and on railroads. Most who develop the disease are men under 50 years
old. The symptoms of silicosis develop about 20 years after initial exposure. Some affected
individuals are asymptomatic, whereas those with more severe disease present with cough,
dyspnea, and increased sputum (black sputum in coal workers).
On chest radiographs, the earliest and most characteristic findings are small, well-circum-
scribed round nodules that predominantly involve the posterior portions of the upper lungs
and are likely to calcify. Hilar and mediastinal lymphadenopathy are common; characteristic
peripheral eggshell calcification of the enlarged nodes (5% of cases) is virtually pathogno-
monic of silicosis. The parenchymal pulmonary nodules increase in size and number as the
disease advances, eventually forming conglomerate masses (> 1 cm) that are usually irregular
and ill defined with peripheral stranding. This process is usually symmetric and located in the
upper lobes or superior segments of the lower lobes and tends to progressively migrate toward
the hilum as the fibrotic process progresses. The volume of the lungs progressively decreases
and cavitation can occur.
On thin-section CT, silicosis typically appears as fine nodular opacifications (1–10 mm)
that are diffusely scattered throughout both lungs in a centrilobular and subpleural location
(Fig. 6). In mild disease, they may be seen only in the upper lobes and have a posterior pre-
dominance. The nodules calcify in about 3% of cases, and chains of subpleural nodules may
produce pseudoplaques. Unlike lymphangitic spread of carcinoma and sarcoidosis, the nod-
ules in silicosis infrequently occur in relation to thickened interlobular septa. The develop-
ment of progressive massive fibrosis, indicating the presence of complicated disease, is al-
ways associated with a background of small nodules visible on thin-section CT.

-ALIGNANCY
Hematogenous Metastases
Pulmonary metastases occur in 20–30% of malignancies, usually the result of hematoge-
nous spread of tumor cells. Less commonly, they are secondary to lymphatic spread. Endo-
bronchial spread is unusual but can occur with head and neck malignancies as well as renal
and breast carcinomas.
On chest radiographs, pulmonary metastases most commonly produce smooth nodules
with well-defined margins that predominantly involve the lower lobes. Depending on their
mode of growth, they can vary from diffuse micronodular shadows resembling military dis-
ease (especially the snowstorm of thyroid carcinoma) to multiple large, well-defined “can-
nonballs” (primarily with choriocarcinoma and seminoma). Cavitation occurs in about 4% of
metastases, most commonly from squamous cell neoplasms (also adenocarcinomas of the
large bowel and sarcomas).

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Boitsios et al.

On CT, hematogenous metastases typi-


cally appear as small discrete nodules that
have a peripheral and basal predominance
when limited in number but a uniform distri-
bution when there are innumerable lesions
(Figs. 7 and 8). Some nodules may appear to
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be related to small branches of pulmonary


vessels. Lymphangitic metastases, which are
thought to be most often of hematogenous
origin with secondary growth into the lym-
phatic system, produce smooth or nodular
thickening of the peribronchovascular inter-
Fig. 7—Metastases from prostate cancer. Two stitium and interlobular septa with preserva-
relatively uniform metastases are seen in right and tion of normal lung architecture at the lobu-
left lung (arrows). lar level (Fig. 9). Hilar lymphadenopathy
occurs in approximately 50% of cases.

Lymphoma
Lymphoma most commonly presents as painless cervical or supraclavicular lymphade-
nopathy. Cough or chest pain may reflect mediastinal involvement, and splenomegaly is often
evident. Today, about 90% of patients with lymphoma are cured of the disease.
One manifestation of secondary lymphoma is multiple nodules, which often have fuzzy out-
lines and are most numerous in the lower lobes. This appearance is usually associated with medi-
astinal and hilar lymph node enlargement. Cystlike lesions may simulate central cavitation.

Bronchioloalveolar Carcinoma
Bronchioloalveolar carcinoma (BAC) is a subtype of adenocarcinoma of the lung that is dis-
tinguished by its peripheral location, well-differentiated cytology, growth along intact alveolar
septa (lepidic growth pattern), and tendency for both bronchial and lymphatic spread. BAC is
divided histologically into mucinous, nonmucinous, and mixed subtypes. Distinguishing muci-
nous from nonmucinous subtypes has significant therapeutic and prognostic implications.
BAC is three to four times more likely to develop in smokers, especially heavy smokers with
long-term exposure. Nevertheless, about one third of cases occur in individuals who have never
smoked and a similar number are former or intermittent smokers. Other postulated risk factors
for BAC include fibrotic scarring in the lung, occupational exposure, and viral infection.
Multiple discrete small nodules, either randomly distributed or primarily centrilobular, in
BAC may mimic hematogenous metastases (Fig. 10). A more common appearance of this con-
dition is diffuse, patchy, or multifocal areas of consolidation that are peribronchovascular and
contain air bronchograms or air-filled cystic spaces. Because fluid and mucus produced by the
tumor are of low attenuation, a characteristic appearance in BAC is the “CT angiogram sign,”
in which contrast-enhanced pulmonary vessels appear denser than surrounding opacified lung.

A B
Fig. 8—Metastases from renal cell cancer.
A, CT image obtained at level of carina shows nodules in both central and peripheral lung parenchyma (arrows).
B, CT image obtained at lower lobe base shows tiny metastases in very peripheral location (arrows).

W358 AJR:194, May 2010


Diffuse Pulmonary Nodules

In addition to these manifestations of BAC, it


should not be forgotten that early adenocarci-
noma may also present as small pulmonary
nodules (Fig. 11).

Kaposi Sarcoma
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Kaposi sarcoma, the most common AIDS-


related multicentric neoplasm, has a propen-
sity to involve the skin, lymph nodes, gastro-
intestinal tract, and lungs. Kaposi sarcoma
develops in about 15–20% of patients with
AIDS, almost all homosexual or bisexual
men, and pulmonary involvement occurs in
about 20% of them. The most common signs
and symptoms are dyspnea and cough; he-
moptysis may develop in advanced stages.
Irregular (flamed-shaped) and ill-defined
peribronchovascular nodules combined with
peribronchovascular and interlobular septal
thickening, pleural effusions, and lymphade-
nopathy may develop in patients with Kaposi Fig. 9—Pulmonary lymphangitic carcinomatosis.
CT image shows nodular thickening of interlobular
sarcoma. In most cases, the presence of typi- septa (curved arrows) and interlobar fissure (straight
cal nodules on CT and a parahilar distribu- arrows). (Reprinted with permission from Webb
tion of abnormalities allow Kaposi sarcoma WR, Müller NL, Naidich DP. High-resolution CT of the
lung, 3rd ed. Philadelphia, PA: Lippincott Williams &
to be distinguished from other thoracic com- Wilkins, 2001)
plications of AIDS.

2ESPIRATORY "RONCHIOLITIS
Respiratory bronchiolitis (RB) is characterized by distortion of the respiratory bronchioles,
with irregular projections of epithelium and smooth muscle into the lumen. A distinct interstitial
lung disease, known as respiratory bronchiolitis–associated interstitial lung disease (RB–ILB),
has been described in smokers or former smokers and is a histologic reaction to dusty envi-

A B
Fig. 10—Bronchioloalveolar carcinoma. (Reprinted with permission from Webb WR, Müller NL, Naidich DP.
High-resolution CT of the lung, 3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins, 2001)
A, CT image shows areas of consolidation in right lower lobes, ill-defined nodules (some of which appear to be
centrilobular), and multiple small, well-defined nodules.
B, Targeted CT image of left lung shows numerous small nodules, at least some of which show random
distribution similar to hematogenous metastases. Note presence of subpleural nodules.

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Boitsios et al.

ronments. Histologically, there is an accumu-


lation of pigmented macrophages within the
respiratory bronchioles and the surrounding
airspaces, with minimal associated mural in-
flammation. Patients may appear subacutely
with cough and dyspnea in their 30s or 40s;
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less often, they present with pneumothorax,


hemoptysis, or acute respiratory failure.
Chest radiographs are normal in individu-
als with noncomplicated RB and about half
of those with RB–ILD. The remainder of pa-
tients with RB–ILD have normal lung vol-
umes but some thickening of the bronchial
walls and a fine reticulonodular pattern.
On thin-section CT, RB–ILD typically
produces faint micronodular nodules (3–5
mm) and patchy ground-glass opacities that
may be widespread but predominantly tend
Fig. 11—Adenocarcinoma. CT image obtained at level to involve the upper lobes (Fig. 12).
of right lower lobe shows two parenchyma nodules,
both histologically proven to be adenocarcinoma. Infection
One nodule is of soft-tissue density and well defined
Numerous infectious diseases can present
(white arrow). Second nodule is ill-defined and
has heterogeneous texture and less density (black the pattern of multiple pulmonary nodules.
arrow). In septic emboli, which is most commonly

Fig. 12—Respiratory bronchiolitis.


A–C, CT images obtained at various levels of right
upper lobe show centrilobular emphysema (white
arrows). Emphysematous lesions are surrounded
by subtle, ill-defined airspace nodules, typical of
disease (black arrows).
A

B C

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Diffuse Pulmonary Nodules

caused by endocarditis or an infected central


catheter, the nodules are diffusely distributed
and frequently cavitate. A characteristic ap-
pearance is feeding vessels in association
with the peripheral nodules.
In reactivation tuberculosis, innumerable
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tiny, discrete, relatively well-defined nodules


may be diffusely distributed throughout both
lungs, producing a miliary pattern that reflects
endobronchial spread of infection (Fig. 13).
Thin-section CT may detect the presence of
diffuse lung involvement when corresponding
chest radiographs are normal or show only
minimal or limited disease. Multiple small
and larger nodules also may be a feature of
atypical (nontuberculous) mycobacterial in-
fection (Figs. 14 and 15). The presence of Fig. 13—Tuberculosis (endobronchial spread in
small nodules in areas of lung distal to a dom- reactivation disease). CT image shows typical
appearance of numerous, diffuse, poorly defined
inant consolidative focus of infection probably nodules, some of which are perivascular and
results from endobronchial spread. centrilobular. (Reprinted with permission from Webb
A pattern of miliary or larger pulmonary WR, Muller NL, Naidich DP. High-resolution CT of the
nodules may be a manifestation of fungal in- lung, 3rd ed. Philadelphia, PA: Lippincott Williams &
Wilkins, 2001)
fection. The most common cause is histo-
plasmosis, in which the generally round or
oval nodules are well circumscribed and often calcify. Other fungal disorders that can pro-
duce multiple pulmonary nodules include coccidioidomycosis, blastomycosis, and candidia-
sis. More irregular and poorly defined masses may be seen in Pseudomonas infection, and

A B

C D
Fig. 14—Mycobacterium avium intracellulare infection.
A–D, CT images obtained at various lung levels. Arrows show ill-defined, subtle ground-glass nodules (A);
larger partly calcified nodules (B); small peripheral peribronchial nodules (C); and small, dense subpleural
nodules (D).

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A B
Fig. 15—Mycobacterium avium intracellulare infection.
A, CT scan in right upper lobe shows small, dense nodules in peribronchial location.
B, More caudal CT image better depicts peripheral location of nodules.

well-circumscribed cystic masses with the predilection of the periphery of the lower lobes are
a characteristic appearance of Paragonimus westermani. In varicella pneumonia (adults more
than children), there is often a miliary pattern of pulmonary nodules that heal with diffuse
calcifications (as in histoplasmosis). Parasitic diseases that may produce military nodules
include schistosomiasis and filariasis.
A common finding on thin-section CT in infectious lung disease is the tree-in-bud pattern,
in which centrilobular bronchial dilatation and filling by mucus, pus, or fluid resembles a
budding tree The pattern of patchy clusters of small nodules and branching soft-tissue opaci-
ties is generally most pronounced in the lung periphery (within 3–5 mm of the pleural sur-
face) and associated with abnormalities of the larger airways.
Aspergillosis is a fungal infection that often occurs in preexisting cavities caused by tubercu-
losis, fungal disease, or sarcoidosis. The disease develops in immune-compromised patients and
typically presents with cough, fever, chills, dyspnea, and chest pain. A halo or ground-glass
opacity surrounding focal dense parenchymal nodules is a characteristic CT appearance of in-
vasive pulmonary aspergillosis (Fig. 16). The halo and central nodule are reported to reflect,
respectively, a rim of coagulation necrosis or hemorrhage surrounding a central fungal nodule
or infarct.

Small Airway Disease


Small airway disease (SAD) refers to a spectrum of disorders in which inflammation of the
smallest bronchi and bronchioles with minimal emphysema leads to distortion and oblitera-
tion of small airways. Diseases affecting the small airways are difficult to detect by tradi-
tional diagnostic tests. There must be widespread involvement before the appearance of
symptoms and abnormalities on pulmonary function testing or chest radiography. However,
on thin-section CT, changes associated with SAD are accurately detected.
One manifestation of SAD is multiple small nodules, which are often found abundantly
along the bronchovascular bundles and histologically prove to be centered about abnormal
bronchioles (Fig. 17). Individual abnormal regions generally can be separated from other in-
volved areas by normal or near-normal lung parenchyma. However, if the nodules coalesce,
larger consolidations or airspace opacities may occur.

Hypersensitivity Pneumonitis
Also known as extrinsic allergic alveolitis, hypersensitivity pneumonitis refers to a group
of allergic lung diseases that are caused by chronic inhalation of a variety of organic and
chemical antigens that leads to an immunologic response of the lung tissues. The most com-
mon forms are farmer’s lung and bird fancier’s lung.

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Diffuse Pulmonary Nodules

Fig. 16—Invasive pulmonary aspergillosis. CT image


shows multiple pulmonary nodules associated with
halo sign. (Reprinted with permission from Webb
WR, Müller NL, Naidich DP. High-resolution CT of the
lung, 3rd ed. Philadelphia, PA: Lippincott Williams &
Wilkins, 2001)
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A B
Fig. 17—Chronic aspiration.
A, CT image at level of left lower lobe shows moderate bronchiectasis and bronchial wall thickening (arrow),
indicative of chronic airways disease.
B, CT image obtained at basis of left lower lobe shows peribronchial ground-glass nodules (arrow) indicating
presence of concomitant parenchymal disease.

The radiographic findings vary with the stage of the disease. In the acute phase, chest ra-
diographs are normal in 90% of cases. There may be a fine nodular or reticulonodular pattern
or airspace consolidation (primarily in the lower lobes) that is often misdiagnosed as pneu-
monia. In the subacute stage, 90% of chest radiographs are abnormal, showing poorly defined
small nodules or lung opacifications with obscuration of vascular margins. In chronic disease,
the major findings are fibrosis with architectural distortion and volume loss.
On thin-section CT, hypersensitivity pneumonitis appears acutely as small, ill-defined cen-
trilobular nodules and bilateral airspace consolidation (Fig. 18). In the subacute stage, there
are patchy ground-glass opacities with ill-defined centrilobular nodules. Characteristic mo-
saic perfusion generally affects the middle and lower lung. In the chronic stage of hypersen-
sitivity pneumonitis, lung fibrosis produces honeycombing, traction bronchiectasis, and archi-
tectural distortion. There also may be reticular opacities randomly distributed in the peri-

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A B
Fig. 18—Hypersensitivity pneumonitis.
A and B, Transverse (A) and coronal (B) CT images show subtle increase of lung density in upper lobes. In
addition, transverse image shows partly confluent and partly individual small and subtle nodules in lung
parenchyma (arrows).

bronchial and subpleural regions. A combination of these findings can also be seen in early
stages of nonspecific interstitial pneumonia, in which parenchymal nodules and early archi-
tectural distortion are key features (Fig. 19).

-ISCELLANEOUS #ONDITIONS
Many disorders are extremely rare but have been included for the sake of completeness.

Alveolar Microlithiasis
Alveolar microlithiasis is a rare disorder of unknown cause characterized by diffuse bilat-
eral deposition of intraalveolar microliths. Most prevalent in Turkey, which accounts for 33%
of the world’s cases, in half of patients there is a genetic anomaly that is familial autosomal
recessive. Despite radiographic abnormalities, about 70% of patients with alveolar micro-
lithiasis are asymptomatic when the diagnosis is made. These patients later may develop
cough and dyspnea, and slow progression of the disease may eventually result in respiratory
or cardiac failure.

A B

Fig. 19—Nonspecific interstitial pneumonia.


A and B, CT images show nodular component of
disease (arrow).
C, CT image shows ground-glass opacities resulting
from coalescent nodules with early architectural
distortion (arrow).
C

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Alveolar microlithiasis produces diffuse, very fine micronodules of calcific density. A


characteristic appearance on chest radiographs is the black pleura sign due to contrast be-
tween the extreme density of the lung parenchyma on one side of the pleura and the ribs on
the other side.

Amyloidosis
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Amyloidosis represents a heterogeneous group of disorders characterized by abnormal ex-


tracellular accumulation of insoluble fibrillar proteins. Vascular deposition of these substanc-
es leads to fragility and bleeding. Patients may be asymptomatic or present with cough, respi-
ratory insufficiency, hemoptysis, recurrent pneumonia, and involvement of other organs or
tissue (heart, kidney, tongue, gastrointestinal tract, skin, and muscle).
Discrete masses of amyloid may develop in the rare parenchymal form of the disease. Mul-
tiple nodules may cavitate and show calcification or ossification. The nodular parenchymal
form of the disease has a better prognosis than the tracheobronchial (obstructive) or diffuse
interstitial type.

Papillomatosis of the Lung


Papillomatosis is a human papillomavirus infection that involves the larynx in 95% of
cases. Airway dissemination seeding the lung occurs in < 1% of cases, most frequently in
children or young adults, and surgical manipulation of a laryngeal papilloma increases the
likelihood of this complication.
Papillomas of the lung typically obstruct the airways, resulting in peripheral atelectasis and
obstructive pneumonia. A rare manifestation is round, sharply circumscribed nodules that
frequently cavitate (often resembling advanced cystic bronchiectasis).

Polyarteritis
Poorly defined nodules, often associated with patchy consolidations, are a rare manifesta-
tion of polyarteritis. The pulmonary appearance in this condition typically shows progression
and regression of lesions on serial studies, reflecting the appearance of new lesions and the
healing of old ones. The CT angiography visualization of multiple arterial aneurysms in one
or more abdominal organs is considered virtually diagnostic of this disease.

Pulmonary Arteriovenous Fistulas


Pulmonary arteriovenous fistula is an abnormal direct communication between pulmonary
arteries and pulmonary veins, which may be acquired or congenital and produce high blood
flow through the right-to-left shunt. About one third are multiple, and about 50% have he-
reditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). A definitive diagnosis re-
quires identification of the feeding artery and the draining vein.
Pulmonary arteriovenous fistulas appear as sharply defined, round or oval, often slightly
lobulated nodules that predominantly involve the lower lobes. The lesions may change in size
between the Valsalva and the Müller maneuver.

Pulmonary Hemosiderosis
Pulmonary hemosiderosis most commonly affects young individuals and is characterized
by recurrent episodes of diffuse pulmonary hemorrhage without an identifiable cause. A fine
miliary pattern of densely calcified or ossified nodules diffusely scattered throughout the
lungs may develop in patients with long-standing mitral stenosis (or other causes of elevated
left atrial pressure) who have had multiple episodes of hemoptysis.

Pulmonary Varices
Pulmonary varices are characterized by rare congenital or acquired tortuosity and dilatation
of the pulmonary veins just before their entrance into the left atrium. The varicosities change
shape and size with the Valsalva and Müller maneuvers (similar to arteriovenous fistulas).
On imaging, pulmonary varices appear as multiple round, well-defined opacities. On chest
radiographs, they most commonly are seen on lateral images projecting posterior and inferior
to the hilar structures. Anatomically, varices are most often solitary and preferentially in-
volve the right lower lobe pulmonary vein.

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Rheumatoid Necrobiotic Nodules


This rare manifestation of rheumatoid lung disease tends to wax and wane in relation to the
activity of rheumatoid arthritis and the presence of subcutaneous nodules. Rheumatoid lung
nodules may be associated with pneumoconiosis (Caplan syndrome). They generally appear
as smooth, well-circumscribed nodules that predominantly occur in peripheral subpleural
locations. Cavitation is common, producing thick-walled lesions with smooth inner margins.
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A miliary pattern of pulmonary nodules may occur in the early subacute stage of the disease
before the development of the more characteristic diffuse interstitial pulmonary fibrosis.

Wegener Granulomatosis
Wegener disease, now often called “antineutrophil cytoplasmic autoantibody (ANCA)-as-
sociated granulomatous vasculitis,” is a necrotizing granulomatous vasculitis of small to me-
dium vessels without associated infection. Of unknown cause, the classic triad consists of
abnormalities involving the sinuses, lungs, and kidneys.
In the chest, Wegener granulomatosis typically produces round, fairly well-circumscribed
nodules that may simulate metastases. Cavitation (thick-walled with irregular, shaggy inner
margins) develops in approximately half the patients. Rarely, small fine nodules may develop
in combination with larger, more ill-defined densities, representing a diffuse granulomatous
reaction occurring around vessels.

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