A retrospective cohort study of outcome in systemic sclerosis-associated interstitial lung disease
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- 作者：Masaki Okamoto ; Ph. D. ; M. D.a ; okamoto_masaki@med.kurume-u.ac.jp ; Kiminori Fujimoto ; Ph. D. ; M. D.b ; c ; kimichan@med.kurume-u.ac.jp ; Junko Sadohara ; Ph. D. ; M. D.b ; c ; sadojun@gmail.com ; Kiyomi Furuya ; Ph. D. ; M. D.b ; furuya@kyumed.jp ; Shinjiro Kaieda ; Ph. D. ; M. D.a ; kaieda@med.kurume-u.ac.jp ; Tomoya Miyamura ; Ph. D. ; M. D.d ; miyamura@kyumed.jp ; Eiichi Suematsu ; Ph. D. ; M. D.e ; suematsu-e@kyumed.jp ; Yasuhiko Kitasato ; Ph. D. ; M. D.a ; kita-sir1028@jcom.home.ne.jp ; Tomotaka Kawayama ; Ph. D. ; M. D.a ; kawayama_tomotaka@med.kurume-u.ac.jp ; Hiroaki Ida ; Ph. D. ; M. D.a ; ida@med.kurume-u.ac.jp ; Masao Ichiki ; Ph. D. ; M. D.e ; f ; ichiki@kyumed.jp ; Tomoaki Hoshino ; Ph. D. ; M. D.a ; hoshino@med.kurume-u.ac.jp
- 关键词：Systemic sclerosis ; Interstitial lung disease ; Computed tomography ; Immunosuppressant ; Acute exacerbation
- 刊名：Respiratory Investigation
- 出版年：2016
- 出版时间：November 2016
- 年：2016
- 卷：54
- 期：6
- 页码：445-453
- 全文大小：1321 K
- 卷排序：54
文摘
The relationship between the histological pattern and survival in systemic sclerosis-associated interstitial lung disease (SSc-ILD) is unclear. In patients with SSc-ILD, we investigated whether the clinical data obtained by non-invasive examinations could be used for prognostic evaluation, and attempted to clarify whether complicating acute exacerbation (AE) and the selection of pharmacological therapy were associated with survival.MethodsThirty-five patients with SSc-ILD, who had not been diagnosed by surgical lung biopsy were analyzed, retrospectively. The HRCT findings were evaluated by 2 radiologists and classified into “CT-UIP” or “CT-inconsistent with UIP” patterns based on whole lung interpretations. HRCT scores were calculated based on the extent of abnormality evidenced by HRCT. The log-rank test was used to determine variables, including clinical parameters and histories.ResultsTwelve (34%) of the 35 patients died during a median follow-up period of approximately 7.9 years. The log-rank test showed that a higher mortality was associated with higher age, a CT-UIP pattern, a higher score for ground-glass attenuation with traction bronchiectasis on HRCT, and complicating AE, whereas a lower mortality was significantly associated with the use of immunosuppressants. A CT-UIP pattern was significantly associated with a higher incidence of later AE.ConclusionTreatment with immunosuppressants was associated with a longer survival, and complicating AE is a predictor of shortened survival in SSc-ILD patients. Among the clinical parameters determined by non-invasive examinations, a CT-UIP pattern and the extent of fibrotic lesions on HRCT, but not a histological pattern of UIP, may be predictors of shortened survival.