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


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2018-12

AUTHORS

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

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. More... »

PAGES

25

Identifiers

URI

http://scigraph.springernature.com/pub.10.1186/s12890-018-0591-y

DOI

http://dx.doi.org/10.1186/s12890-018-0591-y

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1100711019

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/29382307


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