Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy?

Abstract

Background: Pulmonary emphysema combined with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) occurs more often in smokers but also in never-smokers. This study aimed to describe a new finding characterized by peculiar emphysematous change with SSc-associated ILD (SSc-ILD).

Methods: We conducted a retrospective review of 21 consecutive patients with SSc-ILD diagnosed by surgical lung biopsy and focused on the radio-pathological correlation of the emphysematous change.

Results: Pathological pulmonary emphysema (p-PE) with SSc-ILD was the predominant complication in 16 patients (76.2%) with/without a smoking history, of whom 62.5% were never-smokers. A low attenuation area (LAA) within interstitial abnormality on high-resolution computed tomography (HRCT) was present in 31.3%. Diffusing capacity of the lung for carbon monoxide (D_LCO) was lower, disease extent on HRCT higher, and intimal/medial thickening in muscular pulmonary arteries more common in the patients with p-PE with SSc-ILD. However, forced vital capacity (FVC) was well preserved regardless of whether p-PE was observed. Most SSc-ILD patients had pulmonary microvasculature changes in arterioles (90.5%), venules (85.7%), and interlobular veins (81.0%).

Conclusions: Pulmonary emphysematous changes (LAA within interstitial abnormalities on HRCT and destruction of fibrously thickened alveolar walls) are specific and novel radio-pathological features of SSc-ILD. Peripheral vasculopathy may help to destroy the fibrously thickened alveolar walls, resulting in emphysematous change in SSc-ILD.

Keywords: Emphysematous change; Systemic sclerosis; Vasculopathy.

Fig. 1

HRCT and surgical lung biopsy of a 45-year-old never-smoking woman. a Computed tomography scan shows traction bronchiectasis, reticulation predominantly in the peribronchovascular zone, and a low attenuation area (circle) in the subpleural area of both lower lungs. b Pathologic specimen (H & E staining, × 40) shows fibrotic nonspecific interstitial pneumonia with emphysematous change (grade 2). c At high-power magnification, emphysematous change occurs at the fibrously thickened alveolar walls showing an increase in pores (arrows) (Elastica van Gieson staining, × 100); AD: alveolar duct. d Muscular pulmonary artery shows intimal thickening (grade 1) (Elastica van Gieson staining, × 80). e Pulmonary arteriole lying along the alveolar ducts shows intimal thickening and muscularisation (Elastica van Gieson staining, × 200). f A venule shows intimal thickening and muscularisation (Elastica van Gieson staining, × 200)

Fig. 2

HRCT and surgical lung biopsy of a 70-year-old never-smoking woman. a Computed tomography shows reticulation and a low attenuation area within a ground glass opacity and reticulation (circle). b Pathologic specimen (H & E staining, × 40) shows fibrotic nonspecific interstitial pneumonia with emphysematous change (grade 1). c Vascular intimal and medial thickening of a muscular pulmonary artery (grade 2) (Elastica van Gieson staining, × 100). d Intimal fibrosis of alveolar interstitial vessels (arteriole) and e) (venule) (Elastica van Gieson staining, × 200)

Fig. 3

HRCT and surgical lung biopsy of 66-year-old ex-smoking man. a Computed tomography shows reticulation and a low attenuation area within the interstitial abnormalities (circle). b Pathologic specimen (H & E staining, × 40) shows fibrotic nonspecific interstitial pneumonia with emphysematous change (grade 2). c Intimal fibrosis of alveolar interstitial vessels (arteriole) and d (venule) (Elastica van Gieson staining × 200)

Fig. 4

HRCT and surgical lung biopsy of 58-year-old never-smoking woman. a Computed tomography scan shows traction bronchiectasis, reticulation, and ground-glass opacity predominantly in the peribronchovascular zone of both lower lungs. b Pathologic specimen (H & E staining, × 40) shows fibrotic nonspecific interstitial pneumonia with emphysematous change (grade 2). c Muscular pulmonary artery shows medial hypertrophy (grade 1) (Elastica van Gieson staining, × 100). d Pulmonary venule lying along the alveolar ducts shows no apparent intimal thickening (Elastica van Gieson staining, × 200). e Venule and interlobular vein show intimal thickening (Elastica van Gieson staining, × 200)