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Variables associated with functional improvement in patients with interstitial lung disease and myositis related antibodies: Results from a multicentric Latin American study 与间质性肺病和肌炎相关抗体患者功能改善相关的变量:来自拉丁美洲一项多中心研究的结果
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa5194
M. L. Alberti, V. Wolff, F. Reyes, Ernesto Juarez Leon, V. Leiva, G. Carballo, J. Serrano, M. Mejía, L. Fassola, F. Caro, M. Florenzano, F. Paulin
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引用次数: 0
Should we biopsy one or two lobes? – diagnostic yield and risks of transbronchial lung cryobiopsy 我们应该对一个或两个肺叶进行活检吗?经支气管肺低温活检的诊断率和风险
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.oa1612
Nuno Pereira, D. Machado, I. Sucena, D. Coutinho, C. Nogueira, I. Marques, A. Sanches, Ana Oliveira, J. Almeida, S. Campainha, S. Neves
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引用次数: 1
Utility of myositis related antibodies in interstitial lung disease and suspected autoimmunity 肌炎相关抗体在肺间质性疾病和疑似自身免疫中的应用
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa5189
M. Florenzano, Juan Carlos Maya, F. Reyes, Carolina Cuéllar, Alexis Peralta, V. Balboa, L. Toro, V. Wolff
Introduction: Myositis related antibodies (MRA) are helpful in the study of patients with idiopathic inflammatory myopathies (IIM), but their role in patients with interstitial lung disease (ILD) is unclear. The aim of this study is to assess whether MRA contribute to define diagnosis and / or determine change of treatment in a cohort of patients with ILD and to describe the clinical characteristics associated with a positive MRA. Methods: A panel of 16 MRA was performed on 111 patients with ILD who did not have a clear underlying diagnosis. The clinical baseline characteristics associated with a positive MRA were compared. Results: Half of patients had at least 1 positive MRA. There was a change in diagnosis in 27.9% of the patients and in the treatment of 62.3%. Ro-52 and anti-synthetase antibodies were the most frequent. The main rheumatological diagnoses were Anti-Synthetase syndrome, other IIM and ILD with autoimmune features (IPAF). The clinical characteristics that were associated with having a positive MRA were younger age, Raynaud´s, mechanical hands, anti Ro, and anti La. HRCT pattern of NSIP/OP and the absence of pattern of hypersensitivity showed a tendency to predict a positive MRA (Table 1). Conclusions: In the study of patients with ILD, testing MRA is specially useful in younger patients with some specific clinical features and specific HRCT patterns, and they contribute to change diagnosis and treatment.
肌炎相关抗体(MRA)有助于研究特发性炎症性肌病(IIM)患者,但其在间质性肺疾病(ILD)患者中的作用尚不清楚。本研究的目的是评估MRA是否有助于确定ILD患者队列的诊断和/或决定治疗的改变,并描述与MRA阳性相关的临床特征。方法:对111例基础诊断不明确的ILD患者进行16次MRA检查。比较与MRA阳性相关的临床基线特征。结果:半数患者MRA至少1项阳性。有27.9%的患者改变了诊断,62.3%的患者改变了治疗。Ro-52和抗合成酶抗体最为常见。主要的风湿病诊断为抗合成酶综合征,其他IIM和ILD具有自身免疫性特征(IPAF)。与MRA阳性相关的临床特征为年轻、雷诺氏、机械手、抗Ro和抗La。NSIP/OP的HRCT型和无超敏型倾向于预测MRA阳性(表1)。结论:在ILD患者的研究中,检测MRA对具有某些特定临床特征和特定HRCT型的年轻患者特别有用,有助于改变诊断和治疗。
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引用次数: 0
An observational study on effectiveness of azathioprine in treatment of interstitial lung disease secondary to connective tissue disease 硫唑嘌呤治疗结缔组织病继发间质性肺疾病疗效观察研究
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa4740
M. Saeidinejad, Sandosh Gnanalingam, A. Ashish
Azathioprine (AZA) is common treatment for connective tissue disease (CTD) related interstitial lung disease (ILD) which is reflected in the British Thoracic Society 2008 ILD guideline [1]. However, this is only a grade C evidence and no studies have reported the real-life effectives in this patient subgroup. An observational study of the effectiveness of AZA in CTD ILD was carried out by retrospective data collection of 30 patients seen in CTD – ILD clinic between January 2010 and December 2018. The mean age of patients was 62.79 (+/- 13 SD) years of which 16 (53%) were females with underlying CTDs (table 1). 16 (53%) patients continued AZA for a mean duration of 52 (+/- 18 SD) months. The FVC and DLCO were preserved in this group compared to those who did not [Mean FVC change: -1.630% (CI 0.95 = 0.284) vs -15.767% (CI 0.95 = 0.555), Mean DLCO change: -4.478% (CI 0.95 = 0.302) vs -14.233% (CI 0.95 = 0.689) respectively]. AZA was stopped in 16 patients due to toxicity (16%) or intolerability (27%). 2 were later re-started on AZA with good tolerance. Our single centre experience showed that FVC of patients with CTD related ILD, over a mean 50-month duration of therapy with of AZA, remained stable. This leads us to conclude that AZA is effective in treatment of CTD related ILD but prone to poor tolerability and systemic toxicity. Reference: [1] Wells AU, Hirani N. Interstitial lung disease guideline. Thorax. 2008 Sep 1;63(Suppl 5):v1-58.
硫唑嘌呤(Azathioprine, AZA)是治疗结缔组织病(CTD)相关间质性肺疾病(ILD)的常用药物,这在英国胸科学会2008年ILD指南中有所体现[1]。然而,这只是C级证据,并且没有研究报道该患者亚组的实际疗效。通过回顾性收集2010年1月至2018年12月在CTD - ILD诊所就诊的30例患者的数据,对AZA治疗CTD - ILD的有效性进行了观察性研究。患者的平均年龄为62.79 (+/- 13 SD)岁,其中16例(53%)为患有潜在CTDs的女性(表1)。16例(53%)患者持续AZA治疗的平均时间为52 (+/- 18 SD)个月。与未治疗组相比,该组的FVC和DLCO得以保留[平均FVC变化:-1.630% (CI 0.95 = 0.284) vs -15.767% (CI 0.95 = 0.555),平均DLCO变化:-4.478% (CI 0.95 = 0.302) vs -14.233% (CI 0.95 = 0.689)]。16例患者因毒性(16%)或不耐受性(27%)停用AZA。2名患者后来重新开始服用AZA,耐受性良好。我们的单中心经验表明,CTD相关ILD患者的FVC在平均50个月的AZA治疗期间保持稳定。这使我们得出结论,AZA对治疗CTD相关的ILD是有效的,但容易产生耐受性差和全身毒性。参考文献:[1]王晓明,王晓明。肺间质性疾病指南。胸。2008年9月1日;63(增刊5):v1-58。
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引用次数: 0
Serum biomarkers in SSc-ILD: association with presence, severity and prognosis SSc-ILD的血清生物标志物:与存在、严重程度和预后的关系
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa5198
C. Stock, A. Lauretis, D. Visca, C. Daccord, M. Kokosi, V. Alfieri, V. Kouranos, G. Margaritopoulos, P. George, P. Molyneaux, F. Chua, T. Maher, V. Ong, D. Abraham, C. Denton, A. Wells, E. Renzoni
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引用次数: 1
Nocturnal desaturation predicts mortality in immune-mediated ILDs 夜间去饱和可预测免疫介导ild的死亡率
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa5196
G. Margaritopoulos, A. Proklou, D. Bonet, F. Chua, P. George, M. Kokosi, V. Kouranos, E. Renzoni, K. Antoniou, A. Wells
Introduction: Nocturnal desaturation may contribute to long-term pulmonary vascular stress in interstitial lung disease (ILD). We studied the prevalence and prognostic utility of nocturnal desaturation across ILDs. Methods: ILD patients with overnight oximetry (January 2010-December 2013) were included (n=437; Idiopathic Pulmonary Fibrosis, n=122; Connective Tissue Disease-ILD, n=48; Hypersensitivity Pneumonitis, n=60; Non-specific Interstitial Pneumonia, n=56; unclassifiable, n=27; Sarcoidosis, n=66; other, n=58). Desaturation index (DI) was defined as the number of desaturation events >4%/hr. Results: DI was higher in NSIP and CTD-ILD than in IPF (p=0.03 and p=0.02 respectively). In the whole cohort, DLco and serum BNP predicted mortality (HR 0.95; 95% CI 0.94, 0.96; p Conclusion: DI is more prevalent and it is a stronger predictor of mortality in immune-mediated ILDs than in IPF and may be an important manifestation of these disorders.
夜间去饱和可能导致间质性肺病(ILD)患者的长期肺血管应激。我们研究了ild患者夜间血饱和度的患病率和预后价值。方法:纳入2010年1月至2013年12月间进行夜间血氧测定的ILD患者(n=437;特发性肺纤维化,n=122;结缔组织病- ild, n=48;过敏性肺炎,n=60;非特异性间质性肺炎56例;不可归类的,n = 27个;结节病,n = 66;其他,n = 58)。去饱和指数(DI)定义为去饱和事件数>4%/hr。结果:NSIP和CTD-ILD患者DI高于IPF患者(p=0.03和p=0.02)。在整个队列中,DLco和血清BNP预测死亡率(HR 0.95;95% ci 0.94, 0.96;结论:与IPF相比,DI在免疫介导的ild中更为普遍,是更强的死亡率预测因子,可能是这些疾病的重要表现。
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引用次数: 1
Verification of genetic associations with Scleroderma associated Interstitial Lung Disease 硬皮病相关性间质性肺疾病遗传相关性的验证
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa5197
C. Stock, A. Lauretis, D. Visca, C. Daccord, M. Kokosi, V. Alfieri, V. Kouranos, G. Margaritopoulos, P. George, P. Molyneaux, F. Chua, T. Maher, D. Abraham, C. Denton, V. Ong, A. Wells, E. Renzoni
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引用次数: 0
A comparison of heath-related quality of life in IPF and systemic sclerosis-related ILD IPF与系统性硬化性ILD患者健康相关生活质量的比较
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa4735
T. Sjåheim, Anna-Maria Hoffman-Vold, T. Eagan, Arnt‐Ove Hovden, M. Lund, G. Bjerke, T. Jonassen, O. Johansen, M. Durheim
Introduction: IPF and systemic sclerosis (SSc)-related interstitial lung disease (ILD) are fibrotic ILDs with a poor prognosis. Assessment of health-related quality of life (HRQL) is important to understand disease trajectory and response to therapy in ILD; however, few studies have compared HRQL in these conditions. Objective: To compare HRQL in SSc-ILD and IPF, independent of lung function. Methods: The Kings Brief Interstitial Lung Disease Questionnaire (K-BILD), validated to assess HRQL in IPF and scored 0-100 with higher scores indicating better HRQL, was used to compare HRQL between patients with IPF (n=58) and SSc-ILD (n=29) in a multi-center cross-sectional study. ANCOVA was used to compare mean K-BILD score between the IPF and SSc-ILD groups, with adjustment for age, gender, FVC and DLCO. Values are presented as mean (SD). Results: Demographics and lung function are listed in table 1. K-BILD score was 69.4 (17.9) among patients with IPF and 80.5 (21.2) among those with SSc-ILD (p =0.014), indicating slightly better HRQL in SSc-ILD. After covariate adjustment, however, no statistical difference in K-BILD was observed between patients with IPF (70.5 [19.3]) and SSc-ILD (79.8 [21.2], p = 0.096). Conclusions: K-BILD scores appeared to be similar between patients with IPF and SSc-ILD after adjusting for age, gender and lung function. This new observation underscores the impact of lung function impairment on HRQL in ILD and the need for treatments that positively impact HRQL by preserving lung function.
IPF和系统性硬化症(SSc)相关的间质性肺疾病(ILD)是预后较差的纤维化性ILD。健康相关生活质量评估(HRQL)对于了解ILD的疾病轨迹和治疗反应非常重要;然而,很少有研究比较这些情况下的HRQL。目的:比较独立于肺功能的SSc-ILD和IPF的HRQL。方法:在一项多中心横断面研究中,使用king简短间质性肺病问卷(K-BILD)来比较IPF (n=58)和SSc-ILD (n=29)患者的HRQL,该问卷的评分为0-100分,评分越高HRQL越好。采用ANCOVA比较IPF组和SSc-ILD组的平均K-BILD评分,并校正年龄、性别、FVC和DLCO。数值以平均值(SD)表示。结果:人口统计学和肺功能列于表1。IPF患者的K-BILD评分为69.4(17.9)分,SSc-ILD患者的K-BILD评分为80.5(21.2)分(p =0.014), SSc-ILD患者的HRQL略好。但协变量调整后,IPF患者(70.5[19.3])与SSc-ILD患者(79.8 [21.2],p = 0.096) K-BILD无统计学差异。结论:在调整年龄、性别和肺功能后,IPF患者和SSc-ILD患者的K-BILD评分相似。这一新的观察结果强调了肺功能损害对ILD患者HRQL的影响,以及通过保留肺功能来积极影响HRQL的治疗的必要性。
{"title":"A comparison of heath-related quality of life in IPF and systemic sclerosis-related ILD","authors":"T. Sjåheim, Anna-Maria Hoffman-Vold, T. Eagan, Arnt‐Ove Hovden, M. Lund, G. Bjerke, T. Jonassen, O. Johansen, M. Durheim","doi":"10.1183/13993003.congress-2019.pa4735","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa4735","url":null,"abstract":"Introduction: IPF and systemic sclerosis (SSc)-related interstitial lung disease (ILD) are fibrotic ILDs with a poor prognosis. Assessment of health-related quality of life (HRQL) is important to understand disease trajectory and response to therapy in ILD; however, few studies have compared HRQL in these conditions. Objective: To compare HRQL in SSc-ILD and IPF, independent of lung function. Methods: The Kings Brief Interstitial Lung Disease Questionnaire (K-BILD), validated to assess HRQL in IPF and scored 0-100 with higher scores indicating better HRQL, was used to compare HRQL between patients with IPF (n=58) and SSc-ILD (n=29) in a multi-center cross-sectional study. ANCOVA was used to compare mean K-BILD score between the IPF and SSc-ILD groups, with adjustment for age, gender, FVC and DLCO. Values are presented as mean (SD). Results: Demographics and lung function are listed in table 1. K-BILD score was 69.4 (17.9) among patients with IPF and 80.5 (21.2) among those with SSc-ILD (p =0.014), indicating slightly better HRQL in SSc-ILD. After covariate adjustment, however, no statistical difference in K-BILD was observed between patients with IPF (70.5 [19.3]) and SSc-ILD (79.8 [21.2], p = 0.096). Conclusions: K-BILD scores appeared to be similar between patients with IPF and SSc-ILD after adjusting for age, gender and lung function. This new observation underscores the impact of lung function impairment on HRQL in ILD and the need for treatments that positively impact HRQL by preserving lung function.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"44 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122451906","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Effects of nintedanib in patients with systemic sclerosis-associated ILD (SSc-ILD) and differing extents of lung fibrosis: the SENSCIS trial 尼达尼布对系统性硬化症相关ILD (SSc-ILD)和不同程度肺纤维化患者的影响:SENSCIS试验
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa5193
G. Raghu, O. Distler, A. Azuma, Aryeh Fischer, Kristin B. Highland, Masataka Kuwana, Maureen D. Mayes, D. Wachtlin, S. Stowasser, M. Alves, M. Gahlemann, Toby M. Maher
Background: In the SENSCIS trial in patients with SSc-ILD, nintedanib reduced the annual rate of FVC decline (mL/year) vs placebo (primary endpoint). There was no significant difference between groups in change in modified Rodnan skin score (mRSS) or St George’s Respiratory Questionnaire (SGRQ) total score (key secondary endpoints) at week 52. Aim: Assess whether extent of lung fibrosis influenced the efficacy of nintedanib. Methods: Subjects with SSc-ILD, ≥10% fibrosis of the lungs on HRCT and FVC ≥40% predicted were randomised to nintedanib 150 mg bid or placebo. We analysed primary and key secondary endpoints in subgroups with extent of lung fibrosis Results: Mean±SD extent of fibrosis at baseline was 36.8±21.8% in the nintedanib group (n=288) and 35.2±20.7% in the placebo group (n=288); 80.2% and 74.3% of subjects in these groups, respectively, had ≥20% fibrosis. The effect of nintedanib on FVC decline was numerically more pronounced in subjects with ≥20% fibrosis, but the treatment-by-time-by-subgroup interaction did not reach statistical significance. A more pronounced increase in SGRQ total score with nintedanib vs placebo was observed in patients with Conclusion: Nintedanib reduced ILD progression in patients with SSc-ILD irrespective of extent of lung fibrosis at baseline.
背景:在SSc-ILD患者的SENSCIS试验中,与安慰剂相比,尼达尼布降低了FVC年下降率(mL/年)(主要终点)。第52周时,两组患者改良罗德曼皮肤评分(mRSS)或圣乔治呼吸问卷(SGRQ)总分(关键次要终点)的变化无显著差异。目的:评价肺纤维化程度对尼达尼布疗效的影响。方法:SSc-ILD、HRCT上肺纤维化≥10%、FVC预测≥40%的受试者随机分配至尼达尼150mg bid或安慰剂组。我们分析了亚组肺纤维化程度的主要终点和关键次要终点。结果:尼达尼布组(n=288)和安慰剂组(n=288)基线时的平均±SD纤维化程度分别为36.8±21.8%和35.2±20.7%;两组中分别有80.2%和74.3%的受试者有≥20%的纤维化。在纤维化≥20%的受试者中,尼达尼布对FVC下降的影响在数字上更为明显,但按时间按亚组治疗的相互作用没有达到统计学意义。结论:无论基线时肺纤维化程度如何,尼达尼布均可减少SSc-ILD患者的ILD进展。
{"title":"Effects of nintedanib in patients with systemic sclerosis-associated ILD (SSc-ILD) and differing extents of lung fibrosis: the SENSCIS trial","authors":"G. Raghu, O. Distler, A. Azuma, Aryeh Fischer, Kristin B. Highland, Masataka Kuwana, Maureen D. Mayes, D. Wachtlin, S. Stowasser, M. Alves, M. Gahlemann, Toby M. Maher","doi":"10.1183/13993003.congress-2019.pa5193","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5193","url":null,"abstract":"Background: In the SENSCIS trial in patients with SSc-ILD, nintedanib reduced the annual rate of FVC decline (mL/year) vs placebo (primary endpoint). There was no significant difference between groups in change in modified Rodnan skin score (mRSS) or St George’s Respiratory Questionnaire (SGRQ) total score (key secondary endpoints) at week 52. Aim: Assess whether extent of lung fibrosis influenced the efficacy of nintedanib. Methods: Subjects with SSc-ILD, ≥10% fibrosis of the lungs on HRCT and FVC ≥40% predicted were randomised to nintedanib 150 mg bid or placebo. We analysed primary and key secondary endpoints in subgroups with extent of lung fibrosis Results: Mean±SD extent of fibrosis at baseline was 36.8±21.8% in the nintedanib group (n=288) and 35.2±20.7% in the placebo group (n=288); 80.2% and 74.3% of subjects in these groups, respectively, had ≥20% fibrosis. The effect of nintedanib on FVC decline was numerically more pronounced in subjects with ≥20% fibrosis, but the treatment-by-time-by-subgroup interaction did not reach statistical significance. A more pronounced increase in SGRQ total score with nintedanib vs placebo was observed in patients with Conclusion: Nintedanib reduced ILD progression in patients with SSc-ILD irrespective of extent of lung fibrosis at baseline.","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"4 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128914716","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Utility of inhalation challenge test using avian egg for hypersensitivity pneumonia 禽蛋吸入激射试验在过敏性肺炎中的应用
Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa5191
R. Okuda, T. Katano, M. Asaoka, Y. Uchida, S. Ikeda, R. Ootoshi, E. Tabata, R. Shintani, H. Okabayashi, T. Niwa, T. Oda, A. Sekine, H. Kitamura, T. Baba, S. Komatsu, E. Hagiwara, T. Ogura
{"title":"Utility of inhalation challenge test using avian egg for hypersensitivity pneumonia","authors":"R. Okuda, T. Katano, M. Asaoka, Y. Uchida, S. Ikeda, R. Ootoshi, E. Tabata, R. Shintani, H. Okabayashi, T. Niwa, T. Oda, A. Sekine, H. Kitamura, T. Baba, S. Komatsu, E. Hagiwara, T. Ogura","doi":"10.1183/13993003.congress-2019.pa5191","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa5191","url":null,"abstract":"","PeriodicalId":178396,"journal":{"name":"ILD/DPLD of known origin","volume":"3 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2019-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114360390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
期刊
ILD/DPLD of known origin
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