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

Hideaki Yamakawa,Tae Iwasawa,Hideya Kitamura,Tomohisa Baba,Kazuyoshi Kuwano,Tamiko Takemura,Akimasa Sekine,Yumie Yamanaka,Takashi Ogura,Shinichiro Iso,Koji Okudela,Satoshi Ikeda

doi:10.1186/s12890-018-0591-y

Abstract

BackgroundPulmonary 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).MethodsWe 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.ResultsPathological 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%).ConclusionsPulmonary 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.

Highlights

Pulmonary emphysema combined with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) occurs more often in smokers and in never-smokers
This study presented that pulmonary emphysematous changes (LAA within interstitial abnormalities on high-resolution computed tomography (HRCT) and destruction of fibrously thickened alveolar walls) are specific and novel radio-pathological features of SSc-associated ILD (SSc-ILD)
Our analysis emphasizes that the emphysematous change occurring with SSc-ILD is different from the usual form of smokingrelated emphysema, which presents as destructive holes in the center of the secondary lobules and is generally well demarcated from the adjacent parenchyma and which centers around the respiratory bronchioles and alveolar ducts [28]

Summary

Introduction

Pulmonary emphysema combined with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) occurs more often in smokers and in never-smokers. Interstitial lung disease (ILD) is common in patients with systemic sclerosis (SSc), and a minority of patients evolve to end-stage respiratory insufficiency [1]. We previously reported that SSc-related autoantibodypositive ILD, but not CTD, is a different disease entity from that of SSc or mixed CTD associated with ILD [9]. Pathological pulmonary emphysema (p-PE) with ILD was observed more frequently (56%) in the patients with SSc or mixed CTD associated with ILD. A high proportion (64.3%) of these patients were never-smokers [9], and another recent report found that pulmonary emphysema combined with SSc-ILD was present in some never-smokers [10]. Overbeek et al attached importance to the correlation between vasculopathy and ILD in the patients with SSc [11]

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