Pulmonary emphysema not combined with lung fibrosis in systemic sclerosis

Respir Med. 2019 Nov-Dec:160:105816. doi: 10.1016/j.rmed.2019.105816. Epub 2019 Nov 9.


Interstitial Lung Disease (ILD) is a common finding of Systemic Sclerosis (SSc) mainly presenting in the form of Nonspecific Interstitial Pneumonia (NSIP) and deeply affecting patients' prognosis. Beside NSIP, other types of ILD have been reported. The most recently described pattern is the so-called Combined-pulmonary emphysema and lung fibrosis, characterized by the coexistence of both upper lobes centrilobular and paraseptal emphysema and lower lobes ILD. We presented three cases of patients with SSc, in which High Resolution Computed Tomography examinations showed emphysema with atypical distribution and radiological presentation, without or with mild signs of fibrosing lung disease, that stabilized after immunosuppressive treatment.

Keywords: Emphysema; High Resolution Computed Tomography; Interstitial lung disease; Systemic Sclerosis.

MeSH terms

  • Humans
  • Idiopathic Interstitial Pneumonias / complications
  • Immunosuppressive Agents / therapeutic use
  • Pulmonary Emphysema / complications*
  • Pulmonary Emphysema / diagnostic imaging
  • Pulmonary Emphysema / drug therapy
  • Pulmonary Fibrosis / complications*
  • Scleroderma, Systemic / complications*
  • Tomography, X-Ray Computed


  • Immunosuppressive Agents