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Review

Managing interstitial lung disease detected on CT during lung cancer screening

Brian D. Southern, MD, Rachel G. Scheraga, MD and Ruchi Yadav, MD
Cleveland Clinic Journal of Medicine January 2016, 83 (1) 55-65; DOI: https://doi.org/10.3949/ccjm.83a.14157
Brian D. Southern
Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH
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  • For correspondence: [email protected]
Rachel G. Scheraga
Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH
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Ruchi Yadav
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  • FIGURE 1
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    FIGURE 1

    In a 36-year-old woman with an 18-pack-year smoking history, high-resolution CT shows respiratory bronchiolitis in the upper lungs, with bilateral centrilobular ground-glass nodules (red arrow), patchy ground-glass opacities (white solid arrow) and bronchial wall thickening (white dashed arrow).

  • FIGURE 2
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    FIGURE 2

    In a 40-year-old woman with a 15-pack-year smoking history with respiratory bronchiolitis-interstitial lung disease, high-resolution CT obtained through the upper lungs at initial diagnosis (left) shows diffuse faint centrilobular ground-glass nodules (red arrows). High-resolution CT 1 year after smoking cessation (right) shows complete resolution of the centrilobular nodules.

  • FIGURE 3
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    FIGURE 3

    In a 49-year-old woman with a 23-pack-year history of smoking, high-resolution CT obtained through the lower lungs shows features of desquamative interstitial pneumonia: diffuse ground-glass opacities predominantly in the mid-lower lung. No traction bronchiectasis or architectural distortion is seen.

  • FIGURE 4
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    FIGURE 4

    In a 38-year-old man with a 20-pack-year history of smoking, coronal image reformatting shows features of desquamative interstitial pneumonia and emphysema, ie, diffuse distribution of ground-glass opacities with cystic airspaces representing emphysema (red arrow).

  • FIGURE 5
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    FIGURE 5

    In a 46-year-old woman with a 26-pack-year history of smoking, a coronal reformatted image shows various-sized cysts (solid white arrows) and nodules (dashed white arrow) in the upper and midlung zones—a characteristic imaging pattern of smoking-related pulmonary Langerhans cell histiocytosis, which was later confirmed with biopsy in this patient.

  • FIGURE 6
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    FIGURE 6

    Early-stage pulmonary Langer-hans cell histiocytosis (nodule-predominant) mimics metastasis in a 56-year-old woman with a 19-pack-year history of smoking. High-resolution CT obtained through the midlungs shows few irregularly marginated nodules (red arrow), one of which is cavitary (white arrow). There is a background of centrilobular emphysema. No cysts are seen. Histologic study confirmed the diagnosis.

  • FIGURE 7
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    FIGURE 7

    In a 49-year-old male smoker with biopsy-confirmed pulmonary Langerhans cell histiocytosis, high-resolution CT obtained through the upper lungs shows numerous well-circumscribed, thin-walled cysts with absence of nodules. This appearance is typical of advanced (“burned out”) pulmonary Langerhans cell histiocytosis and is virtually indistinguishable from emphysema on high-resolution CT.

  • FIGURE 8
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    FIGURE 8

    In a 41-year-old woman with a 22-pack-year history of smoking and pulmonary Langerhans cell histiocytosis, high-resolution CT through the midlungs at the time of initial diagnosis (left) shows a few solid (red arrow) and cavitary (white arrow) nodules. The lung bases were spared. No cysts were seen. High-resolution CT 2 years after smoking cessation and oral corticosteroid therapy (right) shows near-complete resolution of the nodules.

  • FIGURE 9
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    FIGURE 9

    In a 53-year-old woman with mild dyspnea, high-resolution CT through the lower lungs shows bilateral symmetric peripheral distribution of ground-glass opacities, a feature of nonspecific interstitial pneumonia.

  • FIGURE 10
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    FIGURE 10

    High-resolution CT through the lower lungs in a 60-year-old woman with nonspecific interstitial pneumonia shows bilateral symmetric peribronchovascular distribution of ground-glass and reticular opacities with traction bronchiectasis.

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    FIGURE 11

    High-resolution CT through the lower lungs in a 45-year-old woman with scleroderma and nonspecific interstitial pneumonia shows bilateral symmetric peripheral ground-glass attenuation with reticular opacities, traction bronchiectasis (white solid arrow), traction bronchiolectasis (white dashed arrows), and subpleural sparing. A dilated esophagus (red arrow) correlates with the history of scleroderma.

  • FIGURE 12
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    FIGURE 12

    In a 52-year-old woman with mild dyspnea and fatigue, high-resolution CT through the lower lungs shows bilateral symmetric peripheral ground-glass opacities with sparing of the immediate subpleural lung (white arrows), the most specific sign of nonspecific interstitial pneumonia.

  • FIGURE 13
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    FIGURE 13

    In a 68-year-old man, high-resolution CT through the lower lungs shows features of usual interstitial pneumonia: bilateral subpleural reticular opacities, traction bronchiectasis (blue arrow), ground-glass opacities (red arrow), and honeycombing, seen as rows of clustered subpleural cystic air spaces (yellow arrow).

  • FIGURE 14
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    FIGURE 14

    In a 67-year-old man with usual interstitial pneumonia, high-resolution CT through the lower lungs shows basal-predominant subpleural honeycombing (red arrow). Morphologic heterogeneity is seen with areas of fibrosis immediately adjacent to normal lung (white double arrow).

  • FIGURE 15
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    FIGURE 15

Tables

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    TABLE 1

    Features of nonfibrotic smoking-related interstitial lung disease

    Respiratory bronchiolitisRespiratory bronchiolitis interstitial lung diseaseDesquamative interstitial pneumoniaPulmonary Langerhans cell histiocytosis
    Zonal distributionUpper lobeUpper lobeLower lobe (60%)
    Diffuse (20%)
    Patchy (20%)
    Upper lobe (spares the costophrenic angles)
    Clinical findingsAbsentCough and dyspneaCough and dyspneaCough and dyspnea, constitutional symptoms (1/3 of patients) and pneumothorax (15%)
    Findings on high-resolution computed tomographyPoorly defined centrilobular ground-glass nodulesPoorly defined centrilobular ground-glass nodules
    Patchy ground-glass opacities
    Bronchial wall thickening
    Reticulation occasionally (no traction bronchiectasis or honeycombing)
    Ground-glass opacity (widespread, bilateral and symmetrical in 86%)
    Reticular opacities (59%)
    Traction bronchiectasis
    Honeycombing is uncommon (< 1/3 of patients)
    Cysts: bizarrely shaped and nonuniform in size
    Nodules: irregular or cavitary, centrilobular and peribronchial in location
    Associated featuresCentrilobular emphysema
    Air-trapping
    Centrilobular emphysema
    Peripheral cystic spaces (dilated bronchioles and alveolar ducts)
    Characteristic imageFigure 1Figure 2Figures 3 and 4Figures 5–8
    Typical clinical courseUsually asymptomatic; may progress to respiratory bronchiolitis-interstitial lung disease if smoking continuesCough and progressive dyspnea on exertionCough and progressive dyspnea; can progress despite smoking cessationFever, weight loss, nonproductive cough, dyspnea, chest pain; spontaneous pneumothorax also seen
    Role of lung biopsyNoneNoneSurgical lung biopsy may be indicated if diagnosis is uncertainSurgical lung biopsy may be indicated if diagnosis is uncertain
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    TABLE 2

    Features of fibrotic smoking-related interstitial lung disease

    Nonspecific interstitial pneumoniaUsual interstitial pneumonia, idiopathic pulmonary fibrosis
    Craniocaudal distributionBasilar, symmetricApicobasal gradient
    Axial distributionSubpleural and peribronchovascularSubpleural
    MorphologyGround-glass opacities (80%)
    Subpleural sparing (20%–50%)—most specific
    Reticulation (fine or coarse)
    Traction bronchiectasis
    Consolidation
    Honeycombing (uncommon, 1%–5%)
    Reticulation (coarse)
    Honeycombing (up to 70%)
    Traction bronchiectasis
    Ground-glass opacities (in regions of fibrosis, less extensive than the reticulation)
    Architectural distortion
    Characteristic imageFigures 9–12Figures 13 and 14
    Typical clinical courseProgressive dyspnea, cough, and hypoxemia; often have extrapulmonary manifestations (eg, joint pain, rash, Raynaud phenomenon); may respond to immunosuppressive therapy, especially if ground-glass opacities are presentProgressive dyspnea, cough, and hypoxemia; newly approved antifibrotic agents may slow progression of disease
    Role of lung biopsySurgical lung biopsy may be indicated if diagnosis is uncertainSurgical lung biopsy may be indicated if diagnosis is uncertain
    • View popup
    TABLE 3

    Findings on CT that strongly suggest fibrotic interstitial lung disease

    Usual interstitial pneumonia (including idiopathic pulmonary fibrosis)
    Basal predominance of honeycombing
    Absence of relative subpleural sparing
    Absence of centrilobular nodules
    Nonspecific interstitial pneumonia
    Relative subpleural sparing
    Absence of lobular areas with decreased attenuation
    Lack of honeycombing
    • Information from Silva CI, Müller NL, Lynch DA, et al. Chronic hypersensitivity pneumonitis: differentiation from idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia by using thin-section CT. Radiology 2008; 246:288–297.

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Cleveland Clinic Journal of Medicine: 83 (1)
Cleveland Clinic Journal of Medicine
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Managing interstitial lung disease detected on CT during lung cancer screening
Brian D. Southern, Rachel G. Scheraga, Ruchi Yadav
Cleveland Clinic Journal of Medicine Jan 2016, 83 (1) 55-65; DOI: 10.3949/ccjm.83a.14157

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Managing interstitial lung disease detected on CT during lung cancer screening
Brian D. Southern, Rachel G. Scheraga, Ruchi Yadav
Cleveland Clinic Journal of Medicine Jan 2016, 83 (1) 55-65; DOI: 10.3949/ccjm.83a.14157
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    • ABSTRACT
    • HOW COMMON IS INTERSTITIAL LUNG DISEASE IN SMOKERS?
    • NONFIBROTIC VS FIBROTIC DISEASE
    • NONFIBROTIC INTERSTITIAL LUNG DISEASES
    • FIBROTIC INTERSTITIAL LUNG DISEASES
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