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An integrated approach in the diagnosis of smoking-related interstitial lung diseases

Antonella Caminati, Alberto Cavazza, Nicola Sverzellati, Sergio Harari
European Respiratory Review 2012 21: 207-217; DOI: 10.1183/09059180.00003112
Antonella Caminati
*U.O. Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe – MultiMedica, Milan, #U.O. di Anatomia Patologica Arcispedale Santa Maria Nuova - IRCCS, Reggio Emilia, and ¶Dipartimento di Scienze Radiologiche, Sezione di Diagnostica per Immagini, Università degli Studi di Parma, Parma, Italy.
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Alberto Cavazza
*U.O. Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe – MultiMedica, Milan, #U.O. di Anatomia Patologica Arcispedale Santa Maria Nuova - IRCCS, Reggio Emilia, and ¶Dipartimento di Scienze Radiologiche, Sezione di Diagnostica per Immagini, Università degli Studi di Parma, Parma, Italy.
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Nicola Sverzellati
*U.O. Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe – MultiMedica, Milan, #U.O. di Anatomia Patologica Arcispedale Santa Maria Nuova - IRCCS, Reggio Emilia, and ¶Dipartimento di Scienze Radiologiche, Sezione di Diagnostica per Immagini, Università degli Studi di Parma, Parma, Italy.
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Sergio Harari
*U.O. Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe – MultiMedica, Milan, #U.O. di Anatomia Patologica Arcispedale Santa Maria Nuova - IRCCS, Reggio Emilia, and ¶Dipartimento di Scienze Radiologiche, Sezione di Diagnostica per Immagini, Università degli Studi di Parma, Parma, Italy.
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  • For correspondence: sharari@ilpolmone.it
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  • Figure 1.
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    Figure 1.

    a) Respiratory bronchiolitis: the intra-alveolar accumulation of finely pigmented macrophages (smoker’s macrophages) (haematoxylin and eosin, 400×). Generally smoker's macrophages have a strict bronchiolocentric distribution (b; haematoxylin and eosin 40×), but occasionally they involve the entire lobule (c; haematoxylin and eosin, 20×). d) Respiratory bronchiolitis in a 63-yr-old heavy smoker who was asymptomatic at the time of computed tomography. The high-resolution computed tomography image shows bilateral poorly defined centrilobular nodules in the upper lobes. e) Occasionally, particularly in desquamative interstitial pneumonia, a relatively large number of eosinophils are present and the limits with chronic eosinophilic pneumonia are sometimes blurred (haematoxylin and eosin, 200×).

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    Figure 2.

    Smoking-related fibrosis with dense acellular fibrosis that frequently surrounds cystic emphysematous spaces, both in a) centrilobular (haematoxylin and eosin, 40×) and b) sub-pleural parenchyma (haematoxylin and eosin, 20×). c) Specific smoking-related interstitial abnormalities in a heavy smoker with dyspnoea. A high-resolution computed tomography image showing mild ground-glass opacity and reticulation circumscribing emphysematous areas.

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    Figure 3.

    a) Pulmonary Langerhans’ cell histiocytosis (PLCH) is characterised by an increased number of CD1a+ Langerhans’ cells in the bronchiolar wall (200×). b) In the early/active phase of PLCH stellate cellular nodules with a bronchiolocentric distribution can be seen (haematoxylin and eosin, 10×). c) A stellate fibrotic nodule suggestive of healed PLCH is present on the left and is associated with an extensive accumulation of smoker's macrophages (haematoxylin and eosin, 20×). d) Classical example of PLCH in its mature phase consisting of multiple bronchiolocentric cellular nodules, some of which are fibrotic and irregularly cavitated (haematoxylin and eosin, 10×). e) A high-resolution computed tomography image at the level of the upper lobes showing numerous irregularly marginated bilateral nodules; a few cysts are visible. f) Chronic PLCH showing stellate scars with paracicatricial emphysema (haematoxylin and eosin, 40×).

Tables

  • Figures
  • Table 1. Main histological lesions due to smoking and corresponding high-resolution computed tomography (HRCT) findings
    HistologyHRCT
    Smoker's macrophages within bronchiolar lumens and surrounding alveolar spaces, mild bronchiolar fibrosis and inflammationHazy centrilobular nodules
    Patchy ground-glass opacity
    Mild distortion of bronchiolar lumens with bronchioloectasia and mucostasisMild bronchial dilatation with wall thickening; mild air-trapping at expiratory computed tomography scanning
    Centrilobular and subpleural emphysemaCentrilobular and paraseptal emphysema
    Dense interstitial fibrosis, frequently surrounding emphysematous holesPeripheral and intralobular reticulation, ground-glass opacity intermingled with emphysematous areas
    Increase in Langerhans’ cells forming stellate bronchiolocentric nodules, some of which are irregularly cavitated and fibroticIll-defined or cavitated centrilobular nodules
  • Table 2. Clinical features of respiratory bronchiolitis-interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP) and pulmonary Langerhans’ cell histiocytosis (PLCH) in comparison with combined pulmonary fibrosis and emphysema (CPFE)
    RB-ILDDIPPLCHCPFE
    Smoking100%90%>90%100% (heavy smokers)
    Age3rd to 5th decade3rd to 5th decade3rd to 4th decade6th to 7th decade
    Sex M:FSlight male dominanceNearly 2:11:1Almost all male
    OnsetInsidiousInsidiousInsidiousInsidious
    Presenting symptomsDyspnoea and coughDyspnoea and coughDyspnoea, cough and acute chest pain (when pneumothorax is present)Dyspnoea and cough
    Systemic involvementNoPossible, rarePossibleNo
    Crackles∼50%60%Usually absent87% basal
    ClubbingRareNearly 50%Exceptional43%
    Pulmonary functionMixed defect or normal; reduced DL,CORestrictive; reduced DL,COObstructive or restrictive; reduced DL,CO (especially in patients with vascular involvement)Sub-normal pulmonary volumes; severe reduction of DL,CO
    Pulmonary hypertensionNoRarePossible47% at diagnosis; between 50% and 90%
    Desaturation on 6MWTNoPossibleRareFrequent
    Spontaneous improvementPossibleOccasionallyPossibleNo
    TreatmentSmoking cessationSmoking cessation and steroidsSmoking cessation and steroids?Smoking cessation
    Response to steroidsGoodGoodFairNone
    PrognosisGoodGoodGoodPoor (5-yr survival is 55%)
    Complete recovery possibleYesYesYesNo
    Correlation with lung cancer or other neoplasmsNoRareFrequentFrequent (near 50%)
    • M: male; F: female; 6MWT: 6-min walk test; DL,CO: diffusing capacity of the lung for carbon monoxide.

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An integrated approach in the diagnosis of smoking-related interstitial lung diseases
Antonella Caminati, Alberto Cavazza, Nicola Sverzellati, Sergio Harari
European Respiratory Review Sep 2012, 21 (125) 207-217; DOI: 10.1183/09059180.00003112

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An integrated approach in the diagnosis of smoking-related interstitial lung diseases
Antonella Caminati, Alberto Cavazza, Nicola Sverzellati, Sergio Harari
European Respiratory Review Sep 2012, 21 (125) 207-217; DOI: 10.1183/09059180.00003112
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    • HISTOLOGICAL–RADIOLOGICAL CORRELATIONS
    • DIAGNOSTIC WORK-UP OF PATIENTS WITH SUSPECTED SMOKING-RELATED ILDS
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  • Role of air pollutants in airway epithelial barrier dysfunction
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