How is pulmonary involvement assessed in scleroderma?

Updated: Sep 09, 2019
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Chest radiography is an insensitive imaging procedure that typically shows only late findings of pulmonary fibrosis, such as increased interstitial markings. [101] High-resolution computerized tomography (HRCT) is highly sensitive for revealing pulmonary involvement. HRCT scanning should be performed every 6 months if active alveolitis or interstitial pulmonary fibrosis is present and every year if these abnormalities are not present. For more information, see Thoracic Scleroderma Imaging.

Pulmonary function testing is important in all patients with systemic sclerosis, although lung volumes may correlate poorly with the extent of interstitial lung disease. Results of pulmonary function testing are ultimately abnormal in 80% of the patients. Pulmonary function tests should be performed every 6 to 12 months to detect early abnormalities indicative of development and/or progression of pulmonary hypertension or pulmonary fibrosis.

Bronchoscopy with bronchoalveolar lavage is used to differentiate active infections from progressive interstitial lung disease. [102, 103]

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