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Scleroderma lung disease

Joshua J. Solomon, Amy L. Olson, Aryeh Fischer, Todd Bull, Kevin K. Brown, Ganesh Raghu
European Respiratory Review 2013 22: 6-19; DOI: 10.1183/09059180.00005512
Joshua J. Solomon
*Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO, #Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado, Aurora, CO, ¶Division of Pulmonary and Critical Care Medicine, University of Washington Medical Centre, Seattle, WA, USA
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Amy L. Olson
*Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO, #Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado, Aurora, CO, ¶Division of Pulmonary and Critical Care Medicine, University of Washington Medical Centre, Seattle, WA, USA
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Aryeh Fischer
*Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO, #Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado, Aurora, CO, ¶Division of Pulmonary and Critical Care Medicine, University of Washington Medical Centre, Seattle, WA, USA
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Todd Bull
*Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO, #Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado, Aurora, CO, ¶Division of Pulmonary and Critical Care Medicine, University of Washington Medical Centre, Seattle, WA, USA
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Kevin K. Brown
*Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO, #Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado, Aurora, CO, ¶Division of Pulmonary and Critical Care Medicine, University of Washington Medical Centre, Seattle, WA, USA
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Ganesh Raghu
*Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Denver, CO, #Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado, Aurora, CO, ¶Division of Pulmonary and Critical Care Medicine, University of Washington Medical Centre, Seattle, WA, USA
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  • For correspondence: graghu@uw.edu
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    Figure 1.

    High-resolution computed tomography from a patient with systemic sclerosis showing basilar predominate reticulation and ground-glass opacities with an absence of significant honeycombing in a pattern consistent with nonspecific interstitial pneumonia. The patient also has an air–fluid level in the oesophagus consistent with scleroderma-associated oesophageal dysfunction.

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    Figure 2.

    High-resolution computed tomography from a patient with systemic sclerosis showing peripheral and basilar predominate reticulation and honeycombing with an absence of significant ground-glass opacities in a pattern consistent with usual interstitial pneumonia. The patient also has an air-filled oesophagus consistent with scleroderma-associated oesophageal dysfunction.

  • Figure 3.
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    Figure 3.

    Histopathology results from a patient with systemic sclerosis and nonspecific interstitial pneumonia showing cellular interstitial infiltrates in a temporally uniform distribution.

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    Figure 4.

    Histopathology results from a patient with systemic sclerosis and usual interstitial pneumonia showing patchy interstitial fibrosis in close proximity to unaffected lung tissue.

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    Figure 5.

    A pulmonary arteriole from a patient with systemic sclerosis-associated pulmonary artery hypertension showing significant medial hypertrophy.

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    Figure 6.

    Extent of interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD. A simple stratification that utilises pulmonary function tests (PFTs) and extent of disease on high-resolution computed tomography (HRCT) to provide discriminatory prognostic information. FVC: forced vital capacity. Reproduced from [28] with permission from the publisher.

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    Figure 7.

    A suggested approach for the long-term follow-up of patients with systemic sclerosis-interstitial lung disease. PFTs: pulmonary function tests; HRCT: high-resolution computed tomography.

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    Figure 8.

    An algorithm for the early diagnosis of pulmonary arterial hypertension in systemic sclerosis. RV: right ventricle; TAPSE: tricuspid annular plane systolic excursion; PAP: pulmonary artery pressure; PA: pulmonary artery; BNP: B-type natriuretic protein; lcSSc: limited cutaneous systemic sclerosis; DL,CO: diffusing capacity of the lung for carbon monoxide; RVH: right ventricular hypertrophy; PO2: oxygen tension; TR: tricuspid regurgitation; CT: computed tomography; PE: pulmonary embolism; PF: pulmonary fibrosis; V′/Q′: ventilation/perfusion ratio; ILD: interstitial lung disease; mPAP: mean PAP; PVR: pulmonary vascular resistance; CCB: calcium channel blockers; WHO: World Health Organization; ETRA: endothelin receptor antagonist; PDE: phosphodiesterase. Reproduced from [110] with permission from the publisher.

Tables

  • Figures
  • Table 1. Pulmonary involvement in systemic sclerosis
    Direct pulmonary involvement
        ILD
        ILD with PH
        PH
        Airways disease
        Pleural involvement
    Indirect pulmonary complications
        Gastro-oesophageal reflux and aspiration
        Infection
        Drug toxicity
        Malignancy
        Respiratory muscle weakness
        Restrictive lung disease from skin involvement
        Secondary to cardiac involvement
    Combination of direct and indirect pulmonary involvement
    Other lung diseases unrelated to systemic sclerosis
        COPD/emphysema
        Asthma
        Pulmonary nodules
    • ILD: interstitial lung disease; PH: pulmonary hypertension; COPD: chronic obstructive pulmonary disease.

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Scleroderma lung disease
Joshua J. Solomon, Amy L. Olson, Aryeh Fischer, Todd Bull, Kevin K. Brown, Ganesh Raghu
European Respiratory Review Mar 2013, 22 (127) 6-19; DOI: 10.1183/09059180.00005512

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Scleroderma lung disease
Joshua J. Solomon, Amy L. Olson, Aryeh Fischer, Todd Bull, Kevin K. Brown, Ganesh Raghu
European Respiratory Review Mar 2013, 22 (127) 6-19; DOI: 10.1183/09059180.00005512
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    • TYPES OF PULMONARY INVOLVEMENT
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  • The efficacy of bedside chest ultrasound
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