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. 2018 Jan 30;18(1):25.
doi: 10.1186/s12890-018-0591-y.

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

Hideaki Yamakawa  1   2 , Tamiko Takemura  3 , Tae Iwasawa  4 , Yumie Yamanaka  5   6 , Satoshi Ikeda  5 , Akimasa Sekine  5 , Hideya Kitamura  5   6 , Tomohisa Baba  5 , Shinichiro Iso  7 , Koji Okudela  8 , Kazuyoshi Kuwano  6 , Takashi Ogura  5
Affiliations

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

Hideaki Yamakawa et al. BMC Pulm Med. .

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 (DLCO) 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.

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Conflict of interest statement

Ethics approval and consent to participate

This retrospective cohort study was approved by the institutional review board of Kanagawa Cardiovascular and Respiratory Center (no. 28–11). The patients’ approval or informed consent was not required for a retrospective review of their records, pursuant to the ethical guidelines of the Japanese Ministry of Health, Labor, and Welfare.; however, the present retrospective study was carried out by the opt-out method of our hospital website.

Consent for publication

Not applicable.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
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
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
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
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)
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