{"title":"结缔组织疾病相关纤维化间质性肺疾病患者的诊断和治疗。","authors":"Bonnie Wang, Vivek Nagaraja","doi":"10.2174/18743064-v17-e230714-2022-26","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Fibrotic interstitial lung disease is an important driver of morbidity and mortality in patients with connective tissue diseases (CTD). Due to the lack of prospective randomized trial data in this population, practice pattern variation exists in the management of patients with CTD.</p><p><strong>Case presentation: </strong>This case series describes three patients, each with a different background of autoimmunity complicated by fibrotic interstitial lung disease (ILD). We review their initial presentations, follow their disease trajectories on currently available treatments, and reference forthcoming clinical trials.</p><p><strong>Conclusion: </strong>Clinical impact or potential implications. Response to immunosuppression and antifibrotic therapy is variable in patients with connective tissue disease-related fibrosing interstitial lung disease. Data from prospective clinical trials and longitudinal registry studies will conceivably provide additional insight into improving care for these patients.</p>","PeriodicalId":39127,"journal":{"name":"Open Respiratory Medicine Journal","volume":"17 ","pages":"e187430642306150"},"PeriodicalIF":0.0000,"publicationDate":"2023-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10507213/pdf/","citationCount":"0","resultStr":"{\"title\":\"Diagnosis and Management of Patients with Connective Tissue Disease-related Fibrosing Interstitial Lung Diseases.\",\"authors\":\"Bonnie Wang, Vivek Nagaraja\",\"doi\":\"10.2174/18743064-v17-e230714-2022-26\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Fibrotic interstitial lung disease is an important driver of morbidity and mortality in patients with connective tissue diseases (CTD). Due to the lack of prospective randomized trial data in this population, practice pattern variation exists in the management of patients with CTD.</p><p><strong>Case presentation: </strong>This case series describes three patients, each with a different background of autoimmunity complicated by fibrotic interstitial lung disease (ILD). We review their initial presentations, follow their disease trajectories on currently available treatments, and reference forthcoming clinical trials.</p><p><strong>Conclusion: </strong>Clinical impact or potential implications. Response to immunosuppression and antifibrotic therapy is variable in patients with connective tissue disease-related fibrosing interstitial lung disease. Data from prospective clinical trials and longitudinal registry studies will conceivably provide additional insight into improving care for these patients.</p>\",\"PeriodicalId\":39127,\"journal\":{\"name\":\"Open Respiratory Medicine Journal\",\"volume\":\"17 \",\"pages\":\"e187430642306150\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-08-15\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10507213/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Open Respiratory Medicine Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.2174/18743064-v17-e230714-2022-26\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2023/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Open Respiratory Medicine Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.2174/18743064-v17-e230714-2022-26","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2023/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
Diagnosis and Management of Patients with Connective Tissue Disease-related Fibrosing Interstitial Lung Diseases.
Background: Fibrotic interstitial lung disease is an important driver of morbidity and mortality in patients with connective tissue diseases (CTD). Due to the lack of prospective randomized trial data in this population, practice pattern variation exists in the management of patients with CTD.
Case presentation: This case series describes three patients, each with a different background of autoimmunity complicated by fibrotic interstitial lung disease (ILD). We review their initial presentations, follow their disease trajectories on currently available treatments, and reference forthcoming clinical trials.
Conclusion: Clinical impact or potential implications. Response to immunosuppression and antifibrotic therapy is variable in patients with connective tissue disease-related fibrosing interstitial lung disease. Data from prospective clinical trials and longitudinal registry studies will conceivably provide additional insight into improving care for these patients.
期刊介绍：
The Open Respiratory Medicine Journal is an Open Access online journal, which publishes research articles, reviews/mini-reviews, letters and guest edited single topic issues in all important areas of experimental and clinical research in respiratory medicine. Topics covered include: -COPD- Occupational disorders, and the role of allergens and pollutants- Asthma- Allergy- Non-invasive ventilation- Therapeutic intervention- Lung cancer- Lung infections respiratory diseases- Therapeutic interventions- Adult and paediatric medicine- Cell biology. The Open Respiratory Medicine Journal, a peer reviewed journal, is an important and reliable source of current information on important recent developments in the field. The emphasis will be on publishing quality articles rapidly and making them freely available worldwide.
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