Atypical lung emphysematous changes in systemic sclerosis

Dear Editor, A 36-year-old woman with systemic sclerosis (SSc) under no drug therapy due to symptom remission presented with a 2-week history of dyspnoea, cough and sore throat. She denied smoking and environmental exposures. Physical examination demonstrated Raynaud’s phenomenon, sclerodactyly, digital ulcers and microstomia. Auscultation revealed crackling in the lung bases. A transthoracic echocardiogram was normal. Chest computed tomography (CT) revealed an atypical pattern of emphysematous disease with a subpleural distribution, predominantly in the lower lobes, not fulfilling the criteria for non-specific interstitial pneumonia (NSIP) or combined pulmonary fibrosis and emphysema (CPFE; Figure 1). Comparison with CT images obtained 6 years previously revealed no substantial modification, suggesting that the dyspnoea had not been caused by the acute exacerbation of interstitial disease. Treatment of the upper-airway infection led to a good outcome, with symptom resolution.Figure 1: (a-c) Axial CT images showing emphysematous areas distributed mainly in the subpleural regions of the lungs. (d and e) Coronal slices obtained using minimum-intensity projection demonstrating the subpleural distribution of the emphysema and a few foci with centrilobular distribution, mostly in the lower lobes, where subtle ground-glass opacities are also present. These changes are not accompanied by traction bronchiectasis (which in the absence of emphysematous findings would suggest the diagnosis of non-specific interstitial pneumonia) and do not meet the criteria for combined pulmonary fibrosis and emphysema, thereby characterizing an atypical pattern of emphysematous disease recently described for systemic sclerosis. CT = computed tomographyInterstitial lung involvement is common in SSc, especially in cases of NSIP, characterized by reticular and ground-glass opacities in the lung periphery, beyond traction bronchiectasis and bronchiolectasis in the fibrotic phase.[1] Emphysema in combination with SSc-related interstitial changes (CPFE) is less frequent and occurs even in non-smokers, although its mechanism has not been clearly defined.[2] Emphysema and fibrosis generally predominate in the upper and lower lobes, respectively. Recently, a new CT pattern for SSc has been described, distinct from that of CPFE; it is characterized by emphysema with an atypical distribution and predominance in the lower lobes, often coalescent, with little or no fibrosing interstitial involvement.[3,4] Patients with this pattern were non-smokers, and other causes of emphysematous disease were ruled out. Such findings may represent emphysema related to atypical combined interstitial lung disease with a non-fibrosing NSIP pattern, a subtype of SSc lung involvement in which emphysematous changes prevail and commence in the lower lobes, while fibrotic alterations are almost absent.[4] Awareness of this pattern may enable proper patient management and follow-up. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.