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544 SECTION 4 Respiratory System

A B
Figure 56.18. Drug-induced (minocycline) pulmonary infiltrate with eosinophilia in an 18-year-old. Axial
CT images show characteristic peripheral consolidations at the time of diagnosis (A) and marked improvement
after 2 weeks of corticosteroid therapy and cessation of the inciting drug (B).

A B
Figure 56.19. Chronic granulomatous disease in a 16-year-old male. Axial CT images show necrotic prevascular
mediastinal lymphadenopathy (A, arrow) and an irregular nodular left upper lobe consolidation (B, long arrow).
Multiple smaller nodules were also present, one seen in the right upper lobe (B, short arrow).

resection, and stem cell transplantation. Prophylactic antibiotics

Chronic Granulomatous Disease
Etiology. Chronic granulomatous disease (CGD) is a rare
inherited immunodeficiency disorder that usually is caused
by a mutation in one of the four genes encoding subunits of
the phagocyte nicotinamide adenine dinucleotide phosphate,
impairing superoxide production and oxidative burst mechanisms
that normally assist in killing fungal and intracellular catalase-
positive bacterial organisms. The lungs are the most common
site of infection. Histologically, granulomatous inflammation
and often necrosis are present, with surrounding chronic inflam-
mation and fibrosis, hence the name chronic granulomatous
disease.1–20,107–111
Imaging. A variety of imaging findings may present in patients
who have chronic, recurrent infections, including consolidation,
ground-glass opacities, tree-in-bud opacities, and centrilobular or
random (even miliary) nodules acutely, with bronchiectasis, septal
thickening, air trapping, abscess formation, fibrosis, cysts, and
honeycombing with long-standing disease. Other common thoracic
findings include mediastinal and/or hilar lymphadenopathy, pleural
thickening, empyema, vertebral or rib osteomyelitis, and chest
wall invasion1–20,107–111 (Fig. 56.19).
Treatment and Follow-up. CGD is treated with lipophilic
antibiotics, antifungal agents, interferon-γ, abscess drainage, surgical
and antifungals are beneficial. Fluorine-18 fluorodeoxyglucose
positron emission tomography is more reliable than CT for
distinguishing between active and dormant disease activity (e-Fig.
56.20). Improved diagnosis and treatment have allowed survival
into adulthood.1–20,107–111
Cystic Fibrosis
Etiology. Cystic fibrosis (CF) is caused by autosomal-recessive
mutations in the CF transmembrane regulator (CFTR) gene. It
is the most common genetic disorder leading to chronic pulmonary
disease in children. Recurrent infections develop, in addition to
numerous extrapulmonary manifestations such as meconium ileus
in infancy. With progressive disease, chronic inflammatory changes
occur that lead to alteration of airway walls with epithelial erosion,
partial replacement of the mucosa by granulation tissue, progressive
airway dilation resulting in bronchiectasis, and fibrotic/obliterative
changes involving the small airways.1–20,112–119
Imaging. Chest imaging in early CF may be normal or show
mild to moderate air trapping and/or bronchiectasis (Fig. 56.21).
In more advanced disease, bronchiectasis that is predominant in
the upper lobes, bronchial wall thickening, centrilobular nodular
and tree-in-bud opacities, and mucus plugging with air trapping

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 CHAPTER 56 Diffuse Lung Disease 545

56

A B
Figure 56.21. Cystic fibrosis in a 12-year-old. Paired inspiratory (A) and expiratory (B) phase axial CT images
from within this patient with mild pulmonary cystic fibrosis show no bronchiectasis but demonstrate mosaic
attenuation from air trapping that is accentuated on the expiratory phase.

occur (Fig. 56.22). A finger-in-glove pattern of mucoid impaction

similar to ABPA may be observed. Because of recurrent infec-
tions, mediastinal and hilar lymphadenopathy often are present.
Bronchial artery hypertrophy and tortuosity may also develop
due to chronic airway inflammation. Both radiographic and CT
CF scoring systems are available and correlate with lung function
although primarily used for research purposes. UTE MR is under
investigation as a radiation-free alternative for CF imaging, and
the addition of hyperpolarized gas is promising for functional
ventilation assessment.1–20,112–119
Treatment and Follow-up. Treatment traditionally has focused
on the managing/preventing the sequelae of the disease. The
standard of care most recently has included oral azithromycin,
inhaled tobramycin, hypertonic saline solution, and dornase alfa
(Pulmozyme), which functions to break down thick secretions.
Additional antibiotics are used depending on the type of infec-
tions present and the resistance pattern. Several novel small
molecules are now approved for patients with specific muta-
tions: ivacaftor (Kalydeco) with at least one G551D mutation
and lumacaftor/ivacaftor (Orkambi) for homozygous F508del
mutations. Targeted gene therapies remain an area of active Figure 56.22. Cystic fibrosis in a 10-year-old female. Axial expiratory
investigation.1–20,112–119 CT image shows multifocal mosaic attenuation indicating air-trapping,
bronchiectasis, bronchial wall, thickening, and mucus plugging most
marked in the lingula, left lower lobe, and right middle lobe.
Pulmonary Disease in Association
With Immunosuppression
Etiology. Immunosuppression predisposes to a variety of pulmonary
infections that may present as diffuse lung disease. Commonly
encountered conditions include medication-related immunosuppression
(organ transplant drug maintenance, high-dose steroids, or chemo- may also be present (Fig. 56.23). There should be a low threshold to
therapy), human immunodeficiency virus (HIV)/acquired immune obtain CT even if radiographs are normal to exclude occult abnormali-
deficiency syndrome (AIDS), and congenital immunodeficiency. Patients ties with potentially dire consequences in these high-risk
are at risk for inoculation by both typical organisms and atypical patients.1–20,120–128
pathogens (e.g., fungal, viral, mycobacterial).1–20,120–128 Treatment and Follow-up. Broad-spectrum coverage is initially
Imaging. Imaging findings are variable depending on the immune advisable, including antibacterial, antifungal, and antiviral agents,
status and offending pathogens. However, certain CT patterns are tailored when the specific pathogen(s) are isolated. If feasible, immu-
associated with specific infections. For example, ground-glass opacity nosuppressive regimens may be weaned (or antiretroviral therapy
is seen in pneumocystis and cytomegalovirus; nodules <1 cm are seen increased for HIV). Opportunistic infections can quickly become
in viral pneumonia; nodules >1 cm, potentially cavitary, and the halo life-threatening, and thus extreme vigilance is needed. Prophylactic
sign (ground-glass opacity surrounding a solid nodule) are typical of coverage may also be warranted in some circumstances (e.g., for
invasive aspergillosis; tree-in-bud nodules (centrilobular with linear pneumocystis in HIV with low CD4 count). In one series of children
branching) are seen in infectious bronchiolitis, including endobronchial with diffuse lung disease, overall mortality in immunocompromised
tuberculosis; and consolidation is typical of bacterial pneumonia. patients was >50% compared with 16.3% for immunocompetent
Mediastinal/hilar lymphadenopathy and extrathoracic manifestations hosts.1–20,120

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