Ana Catarina Duarte, Sofia Alegria, Filipe Vinagre, Filipa Ferreira, Ana Cordeiro
{"title":"皮下注射曲普瑞替尼能否成为治疗系统性硬化症相关间质性肺病患者肺动脉高压的替代方法?","authors":"Ana Catarina Duarte, Sofia Alegria, Filipe Vinagre, Filipa Ferreira, Ana Cordeiro","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>Pulmonary hypertension (PH) is one of the most feared complications of systemic sclerosis (SSc). There are currently specific drugs approved for PH group I (pulmonary arterial hypertension - PAH), but for PH related to lung disease (group III) the use of vasodilators is still controversial and not routinely recommended in patients with non-severe PH. However, SSc-PH-interstitial lung disease (ILD) has a poorer survival compared with SSc-PAH, making the management of these patients a challenge, ideally carried out in a reference centre. Herein we report the case of a a 45-year-old female with systemic sclerosis-myositis overlap syndrome, with documented lung involvement (ILD with fibrotic nonspecific interstitial/organizing pneumonia pattern), who was diagnosed with pre-capillary PH. She started sequential combination vasodilator therapy including parenteric prostanoid, with clinical benefit and without evidence of ILD worsening.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"None"},"PeriodicalIF":1.4000,"publicationDate":"2024-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Can subcutaneous treprostinil be an alternative for treating pulmonary hypertension in patients with systemic sclerosis-related interstitial lung disease?\",\"authors\":\"Ana Catarina Duarte, Sofia Alegria, Filipe Vinagre, Filipa Ferreira, Ana Cordeiro\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Pulmonary hypertension (PH) is one of the most feared complications of systemic sclerosis (SSc). There are currently specific drugs approved for PH group I (pulmonary arterial hypertension - PAH), but for PH related to lung disease (group III) the use of vasodilators is still controversial and not routinely recommended in patients with non-severe PH. However, SSc-PH-interstitial lung disease (ILD) has a poorer survival compared with SSc-PAH, making the management of these patients a challenge, ideally carried out in a reference centre. Herein we report the case of a a 45-year-old female with systemic sclerosis-myositis overlap syndrome, with documented lung involvement (ILD with fibrotic nonspecific interstitial/organizing pneumonia pattern), who was diagnosed with pre-capillary PH. She started sequential combination vasodilator therapy including parenteric prostanoid, with clinical benefit and without evidence of ILD worsening.</p>\",\"PeriodicalId\":29669,\"journal\":{\"name\":\"ARP Rheumatology\",\"volume\":\" \",\"pages\":\"None\"},\"PeriodicalIF\":1.4000,\"publicationDate\":\"2024-02-13\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"ARP Rheumatology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"RHEUMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"ARP Rheumatology","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
Can subcutaneous treprostinil be an alternative for treating pulmonary hypertension in patients with systemic sclerosis-related interstitial lung disease?
Pulmonary hypertension (PH) is one of the most feared complications of systemic sclerosis (SSc). There are currently specific drugs approved for PH group I (pulmonary arterial hypertension - PAH), but for PH related to lung disease (group III) the use of vasodilators is still controversial and not routinely recommended in patients with non-severe PH. However, SSc-PH-interstitial lung disease (ILD) has a poorer survival compared with SSc-PAH, making the management of these patients a challenge, ideally carried out in a reference centre. Herein we report the case of a a 45-year-old female with systemic sclerosis-myositis overlap syndrome, with documented lung involvement (ILD with fibrotic nonspecific interstitial/organizing pneumonia pattern), who was diagnosed with pre-capillary PH. She started sequential combination vasodilator therapy including parenteric prostanoid, with clinical benefit and without evidence of ILD worsening.