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...
| Main Authors: | , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2018-01-01
|
| Series: | BMC Pulmonary Medicine |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s12890-018-0591-y |
| _version_ | 1819266039230234624 |
|---|---|
| author | Hideaki Yamakawa Tamiko Takemura Tae Iwasawa Yumie Yamanaka Satoshi Ikeda Akimasa Sekine Hideya Kitamura Tomohisa Baba Shinichiro Iso Koji Okudela Kazuyoshi Kuwano Takashi Ogura |
| author_facet | Hideaki Yamakawa Tamiko Takemura Tae Iwasawa Yumie Yamanaka Satoshi Ikeda Akimasa Sekine Hideya Kitamura Tomohisa Baba Shinichiro Iso Koji Okudela Kazuyoshi Kuwano Takashi Ogura |
| author_sort | Hideaki Yamakawa |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-23T20:54:56Z |
| format | Article |
| id | doaj.art-4fdc4878707243aa9fc4bb9ef4d5cabf |
| institution | Directory Open Access Journal |
| issn | 1471-2466 |
| language | English |
| last_indexed | 2024-12-23T20:54:56Z |
| publishDate | 2018-01-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pulmonary Medicine |
| spelling | doaj.art-4fdc4878707243aa9fc4bb9ef4d5cabf2022-12-21T17:31:33ZengBMCBMC Pulmonary Medicine1471-24662018-01-011811910.1186/s12890-018-0591-yEmphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy?Hideaki Yamakawa0Tamiko Takemura1Tae Iwasawa2Yumie Yamanaka3Satoshi Ikeda4Akimasa Sekine5Hideya Kitamura6Tomohisa Baba7Shinichiro Iso8Koji Okudela9Kazuyoshi Kuwano10Takashi Ogura11Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory CenterDepartment of Pathology, Japanese Red Cross Medical CenterDepartment of Radiology, Kanagawa Cardiovascular and Respiratory CenterDepartment of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory CenterDepartment of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory CenterDepartment of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory CenterDepartment of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory CenterDepartment of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory CenterDepartment of Radiology, Yokohama Rousai Hospital for Labour Welfare CorporationDepartment of Pathobiology, Yokohama City University Graduate School of MedicineDepartment of Respiratory Medicine, Tokyo Jikei University HospitalDepartment of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory CenterAbstract 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.http://link.springer.com/article/10.1186/s12890-018-0591-ySystemic sclerosisEmphysematous changeVasculopathy |
| spellingShingle | Hideaki Yamakawa Tamiko Takemura Tae Iwasawa Yumie Yamanaka Satoshi Ikeda Akimasa Sekine Hideya Kitamura Tomohisa Baba Shinichiro Iso Koji Okudela Kazuyoshi Kuwano Takashi Ogura Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy? BMC Pulmonary Medicine Systemic sclerosis Emphysematous change Vasculopathy |
| title | Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy? |
| title_full | Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy? |
| title_fullStr | Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy? |
| title_full_unstemmed | Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy? |
| title_short | Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy? |
| title_sort | emphysematous change with scleroderma associated interstitial lung disease the potential contribution of vasculopathy |
| topic | Systemic sclerosis Emphysematous change Vasculopathy |
| url | http://link.springer.com/article/10.1186/s12890-018-0591-y |
| work_keys_str_mv | AT hideakiyamakawa emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT tamikotakemura emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT taeiwasawa emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT yumieyamanaka emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT satoshiikeda emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT akimasasekine emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT hideyakitamura emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT tomohisababa emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT shinichiroiso emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT kojiokudela emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT kazuyoshikuwano emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy AT takashiogura emphysematouschangewithsclerodermaassociatedinterstitiallungdiseasethepotentialcontributionofvasculopathy |