Predictors of acute exacerbation in biopsy-proven idiopathic pulmonary fibrosis |
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| Affiliation: | 1. Department of Respiratory Medicine, Okinawa Chubu Hospital, Okinawa, Japan;2. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan;3. Department of Rheumatology, Teikyo University Chiba Medical Center, Chiba, Japan;4. Department of Radiology, National Defense Medical College, Saitama, Japan;5. Department of Pathology, National Hospital Organization Tokyo National Hospital, Tokyo, Japan;1. Section of General Thoracic Surgery, UC Davis Health, University of California, Davis, Sacramento, California;2. Heart Lung Vascular Center, David Grant USAF Medical Center, Travis AFB, California;1. Department of Respiratory Internal Medicine, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan;2. Department of Surgical Oncology, Research Institute for Radiation Biology and Medicine, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan;1. Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, Japan;2. Division of Pulmonology, Department of Internal Medicine, Severance Hospital, Institute of Chest Diseases, Yonsei University College of Medicine, Republic of Korea;3. Center for Medical Education, Sapporo Medical University School of Medicine, Japan;4. Department of Public Health and Hygiene, University of the Ryukyus Graduate School of Medicine, Japan;5. Division of Pulmonary and Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Republic of Korea;6. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Japan;7. Department of Advanced and Integrated Interstitial Lung Diseases Research, School of Medicine, Toho University, Japan;8. Department of Respiratory Medicine, Tokyo Medical and Dental University, Japan;1. Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan;2. Departments of Medicine and Dermatology, Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA;3. Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India;4. Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA;5. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Japan;6. Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Centre, Yokohama, Japan;7. Department of Internal Medicine, Division of Respirology, Neurology, and Rheumatology, Kurume University School of Medicine, Japan;8. Department of Respiratory Medicine, Getwell Hospital and Research Institute, Nagpur, Maharashtra, India;9. Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China;10. Division of Rheumatology, Huashan Hospital, Fudan University, Shanghai, China;11. Boehringer Ingelheim (China) Investment Co., Ltd, Shanghai, China;12. Boehringer Ingelheim (Schweiz) GmbH, Basel, Switzerland;13. Boehringer Ingelheim International GmbH, Ingelheim, Germany;14. Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan |
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| Abstract: | BackgroundAcute exacerbation (AE) is a major cause of death in patients with idiopathic pulmonary fibrosis (IPF). Current evidence on AE-IPF has been largely based on clinical, rather than pathological, analyses.MethodsWe investigated AE incidence and its predictors using clinical, radiological, and pathological data of patients diagnosed with IPF by multi-disciplinary discussion.This study, a secondary analysis of previous research, included 155 patients with IPF who underwent surgical lung biopsy (SLB). Cumulative AE incidence was evaluated by the Kaplan–Meier method. Predictors of AE-IPF were analyzed with a Fine-Gray sub-distribution hazard model. Sub-analysis was performed using propensity score-matching analysis.ResultsIn this cohort, the median age of the patients was 66 years and the median percent-predicted forced vital capacity was 82.8%. The cumulative AE incidence rates at 30 days and one year post SLB were 1.9% and 7.6%, respectively. On multivariable analysis, a lower percent-predicted diffusing capacity of the lung for carbon monoxide (%DLCO) (hazard ratio 0.98 per 1% increase, P = 0.02) and fibroblastic foci (FF)-present (vs. absent; hazard ratio 3.01, P = 0.04) were independently associated with a higher incidence of AE. The propensity score-matching analysis with adjustment for age, gender, and %DLCO revealed that the cumulative AE incidence rate was significantly higher in the FF-present subgroup than in the FF-absent subgroup (1-year incidence rate, 10.5% vs. 0%, respectively; P = 0.04 by Gray's test).ConclusionsFF and %DLCO were independent predictors of AE in patients with biopsy-proven IPF. FF may be associated with the pathogenesis of AE-IPF. |
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| Keywords: | Acute exacerbation Diffusing capacity of the lung for carbon monoxide Fibroblastic foci Idiopathic pulmonary fibrosis AE" },{" #name" :" keyword" ," $" :{" id" :" kwrd0035" }," $$" :[{" #name" :" text" ," _" :" acute exacerbation CI" },{" #name" :" keyword" ," $" :{" id" :" kwrd0045" }," $$" :[{" #name" :" text" ," _" :" Confidence Interval diffusing capacity of the lung for carbon monoxide forced expiratory volume 1.0 (sec) FF" },{" #name" :" keyword" ," $" :{" id" :" kwrd0075" }," $$" :[{" #name" :" text" ," _" :" fibroblastic foci FVC" },{" #name" :" keyword" ," $" :{" id" :" kwrd0085" }," $$" :[{" #name" :" text" ," _" :" forced vital capacity GAP" },{" #name" :" keyword" ," $" :{" id" :" kwrd0095" }," $$" :[{" #name" :" text" ," _" :" Gender–Age–Physiology HR" },{" #name" :" keyword" ," $" :{" id" :" kwrd0105" }," $$" :[{" #name" :" text" ," _" :" hazard ratio HRCT" },{" #name" :" keyword" ," $" :{" id" :" kwrd0115" }," $$" :[{" #name" :" text" ," _" :" high-resolution computed tomography IIP" },{" #name" :" keyword" ," $" :{" id" :" kwrd0125" }," $$" :[{" #name" :" text" ," _" :" idiopathic interstitial pneumonia ILD" },{" #name" :" keyword" ," $" :{" id" :" kwrd0135" }," $$" :[{" #name" :" text" ," _" :" interstitial lung disease IPF" },{" #name" :" keyword" ," $" :{" id" :" kwrd0145" }," $$" :[{" #name" :" text" ," _" :" idiopathic pulmonary fibrosis IQR" },{" #name" :" keyword" ," $" :{" id" :" kwrd0155" }," $$" :[{" #name" :" text" ," _" :" interquartile range KL-6" },{" #name" :" keyword" ," $" :{" id" :" kwrd0165" }," $$" :[{" #name" :" text" ," _" :" Krebs von den Lungen-6 MDD" },{" #name" :" keyword" ," $" :{" id" :" kwrd0175" }," $$" :[{" #name" :" text" ," _" :" multi-disciplinary discussion mMRC" },{" #name" :" keyword" ," $" :{" id" :" kwrd0185" }," $$" :[{" #name" :" text" ," _" :" modified Medical Research Council dyspnea scale SLB" },{" #name" :" keyword" ," $" :{" id" :" kwrd0195" }," $$" :[{" #name" :" text" ," _" :" surgical lung biopsy UIP" },{" #name" :" keyword" ," $" :{" id" :" kwrd0205" }," $$" :[{" #name" :" text" ," _" :" usual interstitial pneumonia |
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