Masaki Okamoto1, Kiminori Fujimoto2, Junko Sadohara3, Kiyomi Furuya4, Shinjiro Kaieda5, Tomoya Miyamura6, Eiichi Suematsu7, Yasuhiko Kitasato8, Tomotaka Kawayama9, Hiroaki Ida10, Masao Ichiki11, Tomoaki Hoshino12. 1. Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: okamoto_masaki@med.kurume-u.ac.jp. 2. Department of Radiology, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan; Center for Diagnostic Imaging, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: kimichan@med.kurume-u.ac.jp. 3. Department of Radiology, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan; Center for Diagnostic Imaging, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: sadojun@gmail.com. 4. Department of Radiology, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: furuya@kyumed.jp. 5. Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: kaieda@med.kurume-u.ac.jp. 6. Department of Internal Medicine and Rheumatology, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Fukuoka 810-8563, Japan. Electronic address: miyamura@kyumed.jp. 7. Department of Respirology, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Fukuoka 810-8563, Japan. Electronic address: suematsu-e@kyumed.jp. 8. Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: kita-sir1028@jcom.home.ne.jp. 9. Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: kawayama_tomotaka@med.kurume-u.ac.jp. 10. Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: ida@med.kurume-u.ac.jp. 11. Department of Respirology, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Fukuoka 810-8563, Japan; Department of Clinical Research Institute, National Hospital Organization Kyushu Medical Center, 1-8-1 Jigyohama, Fukuoka 810-8563, Japan. Electronic address: ichiki@kyumed.jp. 12. Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan. Electronic address: hoshino@med.kurume-u.ac.jp.
Abstract
BACKGROUND: 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. METHODS: Thirty-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. RESULTS: Twelve (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. CONCLUSION: Treatment 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. Copyright Â
BACKGROUND: 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. METHODS: Thirty-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. RESULTS: Twelve (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. CONCLUSION: Treatment 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. Copyright Â
Authors: Nicholas Landini; Martina Orlandi; Cosimo Bruni; Edoardo Carlesi; Cosimo Nardi; Linda Calistri; Giovanni Morana; Sara Tomassetti; Stefano Colagrande; Marco Matucci-Cerinic Journal: Front Med (Lausanne) Date: 2022-01-27