Rare ILD/DPLD最新文献_第4页

Role of the COX2- PGE2 axis in S. pneumoniae- induced pulmonary fibrosis exacerbation in mice COX2- PGE2轴在肺炎链球菌诱导的小鼠肺纤维化加重中的作用

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1396

Tina Bormann, R. Maus, J. Stolper, M. Moog, L. Knudsen, T. Welte, M. Kolb, U. Maus

引用次数: 0

Psychological predictors of health-related quality of life of interstitial lung disease patients 间质性肺病患者健康相关生活质量的心理预测因素

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1403

Carlos Zepeda-Dominguez, V. Peláez-Hernández, Casandra Pineda-Sanchez, Victoria Pachicano-Romero, A. Orea-Tejeda, A. Ibarra-Fernández, L. Luna-Rodríguez, Arely Martinez-Bautista, D. González-Islas, Karla Leticia Rosales-Castillo, R. Sánchez-Santillán, A. Flores-Vargas, K. Balderas-Muñoz, R. Davila-Ramos, A. Jiménez-Valentín, F. Salgado-Fernández, S. Hernández-López, Yuridia Pilozi-Montiel

引用次数: 0

Early-onset therapy with sirolimus helps to improve prognosis of patients with lymphangioleiomyomatosis (LAM) 西罗莫司早期治疗有助于改善淋巴管平滑肌瘤病(LAM)患者的预后

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.oa2144

M. Klingenberg, J. Freise, J. Fuge, T. Welte

{"title":"Early-onset therapy with sirolimus helps to improve prognosis of patients with lymphangioleiomyomatosis (LAM)","authors":"M. Klingenberg, J. Freise, J. Fuge, T. Welte","doi":"10.1183/13993003.congress-2019.oa2144","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.oa2144","url":null,"abstract":"Introduction: LAM is a rare cystic lung disease that affects almost exclusively young women. Therapy with the mTOR-inhibitor sirolimus has been established approximately in year 2006. Lung transplantation has long been performed in patients with progressive clinical course. In Germany, analysis of clinical and lungfunctional data of a substantial number of patients has not so far been performed. Methods: Retrospectively, data of 48 female patients were followed up regarding transplantation as therapeutic solution at pneumology department of MHH over 2,7 (1,0-8,2) years. Transplant-free survival was shown by Kaplan-Meier-curves, comparing outcomes using log-rank test. Patients that did receive treatment with sirolimus have been compared to those without therapy. Here, differentiation between patients diagnosed with LAM before and after year 2006 has been conducted. Results: Mean age at time of investigation was 51,5 (47-62) years, whereas the mean age at time of diagnosis was 38 (33-47) years. Overall, 18 patients (38%) have undergone lung transplantation. Patients in therapy with sirolimus showed a mean FEV1-decrease of 22ml/year. Patients that did not receive sirolimus showed a mean FEV1-decrease of 74ml/year. Ten years after diagnosis, patients with sirolimus showed a transplant-free survival of 97% and differ with p=0,07 from patients without sirolimus and diagnosis after 2006 (60%) and with p=0,12 from patients without sirolimus and diagnosis before 2006 (57%). Conclusion: In this small cohort of patients with LAM sirolimus seems to help to prevent clinical progression and therefore prolongs time to transplantation.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","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":"131369429","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

A characterisation of patients with Antisynthetase Syndrome> 抗合成酶综合征患者的特征分析

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3672

E. Heiden, V. Titmuss, S. Babu

{"title":"A characterisation of patients with Antisynthetase Syndrome>","authors":"E. Heiden, V. Titmuss, S. Babu","doi":"10.1183/13993003.congress-2019.pa3672","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3672","url":null,"abstract":"Introduction: Antisynthetase syndrome describes a clinically heterogeneous form of idiopathic inflammatory myopathy which is characterised by interstitial lung disease (ILD) and antisynthetase antibodies, in the context of myositis, Raynaud phenomenon, mechanics hands and arthralgia. It has an estimated prevalence of 1/25,000-1/33,000 worldwide and typically affects women twice as commonly as men.1 The ILD clinic in Portsmouth serves as a tertiary referral centre for hospitals across Hampshire and currently looks after nine patients with a confirmed diagnosis of Antisythetase Syndrome. Method: We reviewed all patients with a confirmed diagnosis and characterised their condition. Results: The population consists of four women and five men with an average age at diagnosis of 55 (range 38-67). Autoantibodies to Jo-1 were positive in all patients whilst anti-Ro-52 antibodies were positive in 2 patients. Lung involvement characterised on HRCT was described as organising pneumonia in 8 patients and non-specific interstitial pneumonia (NSIP) in 1 patient. The following systemic symptoms were described; rash (54%) mechanic hands, (44%), Raynaud’s phenomenon (33%), myalgia (33%) and sicca symptoms (22%). The mean FVC pre-treatment was 2.44L and mean TLCO 4.02. Following treatment with a variety of immunosuppressive treatments, the mean FVC was 2.68L and mean TLCO 4.89. There was an average of 12% improvement in FVC and 25% improvement in TLCO, compared with baseline readings. Conclusion: Early, aggressive treatment with immunosuppression is important to provide patients with the best chance of improvement in lung function and outcome. 1 www.orpha.net Accessed 14/2/19","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"9 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":"130116117","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

Analysis of multicentric Castleman’s disease with pulmonary involvement: A single center study 多中心Castleman病伴肺部累及的分析:单中心研究

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3675

M. Amano, Hiroki Ohta, Gen Kida, Y. Tsukahara, Emiri Tsumiyama, K. Kusano, T. Nishizawa, T. Ohba, H. Yamakawa, R. Kawabe, Shintaro Sato, K. Akasaka, H. Matsushima

{"title":"Analysis of multicentric Castleman’s disease with pulmonary involvement: A single center study","authors":"M. Amano, Hiroki Ohta, Gen Kida, Y. Tsukahara, Emiri Tsumiyama, K. Kusano, T. Nishizawa, T. Ohba, H. Yamakawa, R. Kawabe, Shintaro Sato, K. Akasaka, H. Matsushima","doi":"10.1183/13993003.congress-2019.pa3675","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3675","url":null,"abstract":"Background: Multicentric Castleman’s disease (MCD) is a rare polyclonal lymphoproliferative disorder and often involves pulmonary lesions. An infection by a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease in about half of people with MCD. Whilst in Japan, most of the cases are associated with negative HHV-8. There is no standard therapy for MCD, and the literature consists mostly of single case reports and small case series. Aim: To analyze the clinical features and treatment options of MCD. Methods: The medical records of patients with MCD from 2008 to 2018 were retrospectively reviewed. Results: Seven patients (male: 2, female: 5) were diagnosed in those 11 years. All were non - HIV. The average age at diagnosis was 45 years. These 7 patients visited hospital because of: cough in 2, arthralgia in 1, chest pain in 1, fever in 1, anemia in 1, and finally chest X-ray abnormality in 1. Radiological findings revealed centrilobular nodules in 6 (86%), septal thickening in 6 (86%), multiple nodules in all (100%), ground glass attenuation in 5 (71%), and cysts in 4 (57%). Laboratory findings for almost all of the patients revealed anemia and elevated serum levels of CRP, IgG. Five patients were treated with steroid and tocilizumab and the remaining 2 had no treatment. Conclusion: The clinical course of MCD varies from stable to progressive. Long term treatment was needed and the combination of tocilizumab with steroid allowed the steroid to be tapered off safely and to successfully prevent the progression of MCD.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","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":"133976564","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

Vascular endothelial growth factor (VEGF) is a predictable marker for FEV1 progression in patients with Lymphangiolyomyomatosis (LAM) 血管内皮生长因子(VEGF)是淋巴管多肌瘤病(LAM)患者FEV1进展的可预测标志物。

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3685

J. Freise, M. Klingenberg, J. Fuge, T. Welte

{"title":"Vascular endothelial growth factor (VEGF) is a predictable marker for FEV1 progression in patients with Lymphangiolyomyomatosis (LAM)","authors":"J. Freise, M. Klingenberg, J. Fuge, T. Welte","doi":"10.1183/13993003.congress-2019.pa3685","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3685","url":null,"abstract":"Introduction: LAM is a rare multisystem disease in young women. FEV1 decrease is a main complication. Several studies have shown that serum levels of VEGF levels are elevated in up to 2/3 of women with LAM. The key treatment for parenchymal lung disease is sirolimus. We here report FEV1 development in 23 LAM patients according to VEGF levels and how sirolimus affects FEV1 course dependent on VEGF levels in these patients. Methods: Data from 48 LAM patients were followed up on at pneumology of MHH regarding VEGF levels and FEV1 development over 2,7 (1,0-8,2) years. 18 Patients were transplanted and therefore excluded. For 23 non-transplanted Patients VEGF levels were available. For each Patient linear regression was conducted to specify patients FEV1-course in ml/year decrease. Linear regression was conducted to show relation of FEV1 and VEGF, further stratified by sirolimus therapy. Results: Median age of patients was 51,5 (47-62) years, whereas the mean age of time of diagnosis was 38 (37-47) years. VEGF median was 1,2 (0,62-2,23 )pg/ml; IQR of all patients, 0,96 (0,42-2,24) pg/ml; IQR of patients naive to sirolimus and 1,4 (0,71-1,97) pg/ml; IQR of patients treated with sirolimus. FEV1- decrease of all patients was 30,5 (-19,3-97,3) ml/ year; IQR. Patients without treatment showed a mean decrease of app. 24 (23,63) ml/ year in FEV1. Patients treated with sirolimus showed an increase of FEV1 of app. 96 (96,3) ml/ year. Conclusion: Elevated levels of VEGF in patients with LAM are associated with higher loss of FEV1. Treatment with sirolimus however stops FEV1-decrease and leads to improvement of FEV1 in LAM patients.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","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":"115507496","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: 2

Long-term sirolimus treatment in lymphangioleiomyomatosis 西罗莫司长期治疗淋巴管平滑肌瘤病

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3687

Eva Revilla Lopez, C. Berastegui, B. Saez, A. Méndez, M. Meseguer, I. Sansano, Susana Gomez Olles, A. Roman

{"title":"Long-term sirolimus treatment in lymphangioleiomyomatosis","authors":"Eva Revilla Lopez, C. Berastegui, B. Saez, A. Méndez, M. Meseguer, I. Sansano, Susana Gomez Olles, A. Roman","doi":"10.1183/13993003.congress-2019.pa3687","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3687","url":null,"abstract":"Introduction: Lymphangioleiomyomatosis (LAM) is a rare slowly progressive neoplasic disease that leads to respiratory failure. Short-term treatment with siroliumus has shown to stabilize pulmonary function but data on long-term results lack. The aim of this study was to describe the long-term impact of sirolimus treatment. Methods: From November 2007 to October 2018, 46 LAM patients treated with sirolimus from a tertiary referral centre were retrospectively included. Sirolimus response at 1 year was evaluated. A negative response was defined as a decrease in FEV1 greater than 20 mL/year considered to be the physiological age-related decline. The response was revaluated after 5 years of treatment. Results: 11 (24%) from 46 patients were treated with sirolimus for less than 1 year, 35 (76%) for more than 1 year and 24 (52%) for at least 5 years. A positive response to sirolimus at first year was observed in 21 (60%) patients. Mean pulmonary function test values after one year of sirolimus treatment were FVC 3377 (SD 864) mL, FEV1 2221 (SD 862) mL, and DLCO 56% (SD 21), in the responder group and FVC 2763 (SD 960) mL, FEV1 1736 (743) mL and DLCO 47% (SD 16) in the non-responder group (p Conclusion: Our study supports the idea that sirolimus treatment has a positive long-term impact in nearly half of LAM patients. This is the first study that provides pulmonary function values at 1 and 5 years in LAM patients treated with sirolimus.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"188 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":"122111171","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

Effectiveness of corticosteroid in diffuse alveolar hemorrhage with hematologic malignancy 皮质类固醇治疗弥漫性肺泡出血合并血液恶性肿瘤的疗效

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1401

K. M. Song, J. H. Ahn, Y. Koh

引用次数: 0

Transcriptomic profiling of CD4 lymphocytes in a murine model of AdTGFß-1 induced lung fibrosis AdTGFß-1诱导肺纤维化小鼠模型中CD4淋巴细胞的转录组学分析

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa1397

C. Hinze, M. Moog, R. Maus, D. DeLuca, T. Welte, M. Kolb, U. Maus

引用次数: 0

Lung involvement in common variable immunodeficiency (CVID): a case series 常见变异性免疫缺陷(CVID)累及肺部:一个病例系列

Rare ILD/DPLD Pub Date : 2019-09-28 DOI: 10.1183/13993003.congress-2019.pa3677

A. Moreira, C. Lopes, N. Melo, H. Bastos, P. Mota, A. Morais

{"title":"Lung involvement in common variable immunodeficiency (CVID): a case series","authors":"A. Moreira, C. Lopes, N. Melo, H. Bastos, P. Mota, A. Morais","doi":"10.1183/13993003.congress-2019.pa3677","DOIUrl":"https://doi.org/10.1183/13993003.congress-2019.pa3677","url":null,"abstract":"Introduction: CVID fits into the broad spectrum of rare primary immunodeficiencies, with a global incidence of 1: 25,000 to 1: 50,000. About 10-20% of patients have lymphocytic infiltrates and/or sarcoid-like granulomas, with several histological findings, termed granulomatous and lymphocytic interstitial lung disease (GLILD). Organising pneumonia (OP) is a rare pulmonary manifestation. Aims: Here, we present a case series of CVID with different ILD spectra to put in evidence both severity and imaging and histological aspects. Results: Four females and 2 males were included (mean age 42.5±14.2 years; mean age at diagnosis of CVID 38.7±11years). Clinical manifestations included recurrent sinuses and respiratory infections, severe gastrointestinal manifestations and neurological involvement at presentation. ILD and CVID diagnosis was coincident in 4 cases. After lung, the most commonly affected sites were lymph nodes, gastrointestinal tract and skin. Histological diagnosis was obtained by transthoracic lung biopsy in all cases. After multidisciplinary evaluation of clinical, imagiological and histological aspects the final diagnosis was OP (3 cases), GLILD (2 cases) and lymphocytic pneumonia (1 case). All patients were under immunoglobulin replacement. Four patients were under immunosuppressive treatment (mycophenolate mofetil in 2 cases, corticosteroid and hydroxychloroquine, and corticosteroid alone in the others), in 1 due to interstitial involvement and respiratory function impairment, while to other due to extrapulmonary involvement. Discussion: GLILD and OP are relatively unusual CVID complications, still poorly recognized. Additional investigation is needed to better guide therapeutic options.","PeriodicalId":267660,"journal":{"name":"Rare ILD/DPLD","volume":"38 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":"116946956","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