Epoprostenol for Treatment of Pulmonary Hypertension in Patients With Systemic Lupus Erythematosus
Pulmonary hypertension with pathologicalchanges similar to those observed in primary pulmonary hypertensionoccurs in patients with systemic lupus erythematosus (SLE). Theefficacy of chronic epoprostenol therapy in SLE has not been welldescribed. The objective of this paper is to describe our experie...
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creator | Robbins, Ivan M. Gaine, Sean P. Schilz, Robert Tapson, Victor F. Rubin, Lewis J. Loyd, James E. |
description | Pulmonary hypertension with pathologicalchanges similar to those observed in primary pulmonary hypertensionoccurs in patients with systemic lupus erythematosus (SLE). Theefficacy of chronic epoprostenol therapy in SLE has not been welldescribed. The objective of this paper is to describe our experiencewith long-term epoprostenol therapy in patients with pulmonaryhypertension associated with SLE.
Case seriesof six patients with SLE and associated pulmonary hypertensionreceiving chronic treatment with epoprostenol.
All 6 patients had severe pulmonary hypertension.Mean pulmonary artery pressure (mPAP) was 57 ± 9 mm Hg (mean±SD), and pulmonary vascular resistance was 14 ± 7 units beforebeginning therapy with epoprostenol. In 4 patients who underwent repeathemodynamic evaluation (9 to 16 months after starting epoprostenol), mean pulmonary artery pressure decreased by 38 ± 21% andpulmonary vascular resistance by 58 ± 12%. Clinically, allpatients improved from New York Heart Association class III or IV toclass I or II. Doses of epoprostenol ranged from 4 to 46 ng/kg/min, andthe longest duration of therapy has been 2.5 years. Side effects fromepoprostenol have not differed from those seen in patients with primarypulmonary hypertension, and except for one patient, there has been noexacerbation of SLE.
Epoprostenol waseffective for the treatment of pulmonary hypertension in this smallgroup of patients with SLE. Further evaluation of epoprostenol therapyfor patients with SLE and other diseases associated with pulmonaryhypertension is warranted. |
doi_str_mv | 10.1378/chest.117.1.14 |
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Case seriesof six patients with SLE and associated pulmonary hypertensionreceiving chronic treatment with epoprostenol.
All 6 patients had severe pulmonary hypertension.Mean pulmonary artery pressure (mPAP) was 57 ± 9 mm Hg (mean±SD), and pulmonary vascular resistance was 14 ± 7 units beforebeginning therapy with epoprostenol. In 4 patients who underwent repeathemodynamic evaluation (9 to 16 months after starting epoprostenol), mean pulmonary artery pressure decreased by 38 ± 21% andpulmonary vascular resistance by 58 ± 12%. Clinically, allpatients improved from New York Heart Association class III or IV toclass I or II. Doses of epoprostenol ranged from 4 to 46 ng/kg/min, andthe longest duration of therapy has been 2.5 years. Side effects fromepoprostenol have not differed from those seen in patients with primarypulmonary hypertension, and except for one patient, there has been noexacerbation of SLE.
Epoprostenol waseffective for the treatment of pulmonary hypertension in this smallgroup of patients with SLE. Further evaluation of epoprostenol therapyfor patients with SLE and other diseases associated with pulmonaryhypertension is warranted.</description><identifier>ISSN: 0012-3692</identifier><identifier>EISSN: 1931-3543</identifier><identifier>DOI: 10.1378/chest.117.1.14</identifier><identifier>PMID: 10631192</identifier><identifier>CODEN: CHETBF</identifier><language>eng</language><publisher>Northbrook, IL: Elsevier Inc</publisher><subject>Adult ; Antihypertensive Agents - therapeutic use ; Biological and medical sciences ; Cardiac Catheterization ; Cardiovascular disease ; Case-Control Studies ; Complications and side effects ; Congenital diseases ; CREST = variant of sclerodermacharacterized by calcinosis, Raynaud's phenomenon, esophageal motilitydisorders, sclerodactyly, and telangiectasia ; Drug therapy ; epoprostenol ; Epoprostenol - therapeutic use ; Female ; Heart ; Hemodynamics ; Humans ; Hypertension, Pulmonary - drug therapy ; Hypertension, Pulmonary - etiology ; Hypertension, Pulmonary - physiopathology ; Intubation ; Lupus ; Lupus Erythematosus, Systemic - complications ; Medical sciences ; mPAP = mean pulmonaryartery pressure ; Nitric oxide ; PAP = pulmonary artery pressure ; Patients ; Pharmacology. Drug treatments ; PPH = primarypulmonary hypertension ; Pulmonary arteries ; Pulmonary hypertension ; Pulmonary Wedge Pressure - drug effects ; PVR = pulmonary vascular resistance ; Respiratory system ; Scleroderma ; SLE = systemic lupus erythematosus ; Systemic lupus erythematosus ; Treatment Outcome ; Vascular Resistance - drug effects ; Vasodilation - drug effects ; Veins & arteries</subject><ispartof>Chest, 2000-01, Vol.117 (1), p.14-18</ispartof><rights>2000 The American College of Chest Physicians</rights><rights>2000 INIST-CNRS</rights><rights>COPYRIGHT 2000 Elsevier B.V.</rights><rights>Copyright American College of Chest Physicians Jan 2000</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c537t-255844e763daedefe040fc3000ee26a58ceab1af168bf13ec5d1ef43e859df53</citedby><cites>FETCH-LOGICAL-c537t-255844e763daedefe040fc3000ee26a58ceab1af168bf13ec5d1ef43e859df53</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,4010,27900,27901,27902</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1232543$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10631192$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Robbins, Ivan M.</creatorcontrib><creatorcontrib>Gaine, Sean P.</creatorcontrib><creatorcontrib>Schilz, Robert</creatorcontrib><creatorcontrib>Tapson, Victor F.</creatorcontrib><creatorcontrib>Rubin, Lewis J.</creatorcontrib><creatorcontrib>Loyd, James E.</creatorcontrib><title>Epoprostenol for Treatment of Pulmonary Hypertension in Patients With Systemic Lupus Erythematosus</title><title>Chest</title><addtitle>Chest</addtitle><description>Pulmonary hypertension with pathologicalchanges similar to those observed in primary pulmonary hypertensionoccurs in patients with systemic lupus erythematosus (SLE). Theefficacy of chronic epoprostenol therapy in SLE has not been welldescribed. The objective of this paper is to describe our experiencewith long-term epoprostenol therapy in patients with pulmonaryhypertension associated with SLE.
Case seriesof six patients with SLE and associated pulmonary hypertensionreceiving chronic treatment with epoprostenol.
All 6 patients had severe pulmonary hypertension.Mean pulmonary artery pressure (mPAP) was 57 ± 9 mm Hg (mean±SD), and pulmonary vascular resistance was 14 ± 7 units beforebeginning therapy with epoprostenol. In 4 patients who underwent repeathemodynamic evaluation (9 to 16 months after starting epoprostenol), mean pulmonary artery pressure decreased by 38 ± 21% andpulmonary vascular resistance by 58 ± 12%. Clinically, allpatients improved from New York Heart Association class III or IV toclass I or II. Doses of epoprostenol ranged from 4 to 46 ng/kg/min, andthe longest duration of therapy has been 2.5 years. Side effects fromepoprostenol have not differed from those seen in patients with primarypulmonary hypertension, and except for one patient, there has been noexacerbation of SLE.
Epoprostenol waseffective for the treatment of pulmonary hypertension in this smallgroup of patients with SLE. Further evaluation of epoprostenol therapyfor patients with SLE and other diseases associated with pulmonaryhypertension is warranted.</description><subject>Adult</subject><subject>Antihypertensive Agents - therapeutic use</subject><subject>Biological and medical sciences</subject><subject>Cardiac Catheterization</subject><subject>Cardiovascular disease</subject><subject>Case-Control Studies</subject><subject>Complications and side effects</subject><subject>Congenital diseases</subject><subject>CREST = variant of sclerodermacharacterized by calcinosis, Raynaud's phenomenon, esophageal motilitydisorders, sclerodactyly, and telangiectasia</subject><subject>Drug therapy</subject><subject>epoprostenol</subject><subject>Epoprostenol - therapeutic use</subject><subject>Female</subject><subject>Heart</subject><subject>Hemodynamics</subject><subject>Humans</subject><subject>Hypertension, Pulmonary - drug therapy</subject><subject>Hypertension, Pulmonary - etiology</subject><subject>Hypertension, Pulmonary - physiopathology</subject><subject>Intubation</subject><subject>Lupus</subject><subject>Lupus Erythematosus, Systemic - complications</subject><subject>Medical sciences</subject><subject>mPAP = mean pulmonaryartery pressure</subject><subject>Nitric oxide</subject><subject>PAP = pulmonary artery pressure</subject><subject>Patients</subject><subject>Pharmacology. Drug treatments</subject><subject>PPH = primarypulmonary hypertension</subject><subject>Pulmonary arteries</subject><subject>Pulmonary hypertension</subject><subject>Pulmonary Wedge Pressure - drug effects</subject><subject>PVR = pulmonary vascular resistance</subject><subject>Respiratory system</subject><subject>Scleroderma</subject><subject>SLE = systemic lupus erythematosus</subject><subject>Systemic lupus erythematosus</subject><subject>Treatment Outcome</subject><subject>Vascular Resistance - drug effects</subject><subject>Vasodilation - drug effects</subject><subject>Veins & arteries</subject><issn>0012-3692</issn><issn>1931-3543</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2000</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNp1ktuLEzEUxgdR3Lr66qMEEZ-cmstcH5el6woFFyz4GNLMSZtlJqlJZqX__Z46hRap5CEXfuc7ly9Z9p7RORN181VvIaY5Y_WczVnxIpuxVrBclIV4mc0oZTwXVcuvsjcxPlK8s7Z6nV0xWgk88lm2Xuz8LviYwPmeGB_IKoBKA7hEvCEPYz94p8Ke3O93EJCK1jtiHXlQySIUyS-btuTnHhUGq8ly3I2RLMI-bWFQyccxvs1eGdVHeHfcr7PV3WJ1e58vf3z7fnuzzHUp6pTzsmyKAupKdAo6MEALarTAogF4pcpGg1ozZVjVrA0ToMuOgSkENGXbmVJcZ58nWWzn94hTkYONGvpeOfBjlDVt2rrhFMGP_4CPfgwOS5Oc0pI3ONgTtFE9SOuMT0Hpg6K8KVvOKG04Ql8uQBtwEFTvHRiLz-d4fgHH1R1Gd4mfT7xGg2IAI3fBDmiGZFQe3Jd_3ZfovmSSFRjw4djZuB6gO8MnuxH4dARU1Ko3QTlt44njguPPOSXe2s32jw0g46D6HlXFlPI4sLPEzRQA6O-ThSCjxt-hocNgnWTn7f9qfgYyzNy2</recordid><startdate>200001</startdate><enddate>200001</enddate><creator>Robbins, Ivan M.</creator><creator>Gaine, Sean P.</creator><creator>Schilz, Robert</creator><creator>Tapson, Victor F.</creator><creator>Rubin, Lewis J.</creator><creator>Loyd, James E.</creator><general>Elsevier Inc</general><general>American College of Chest Physicians</general><general>Elsevier B.V</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9-</scope><scope>K9.</scope><scope>KB0</scope><scope>M0R</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>200001</creationdate><title>Epoprostenol for Treatment of Pulmonary Hypertension in Patients With Systemic Lupus Erythematosus</title><author>Robbins, Ivan M. ; Gaine, Sean P. ; Schilz, Robert ; Tapson, Victor F. ; Rubin, Lewis J. ; Loyd, James E.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c537t-255844e763daedefe040fc3000ee26a58ceab1af168bf13ec5d1ef43e859df53</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2000</creationdate><topic>Adult</topic><topic>Antihypertensive Agents - therapeutic use</topic><topic>Biological and medical sciences</topic><topic>Cardiac Catheterization</topic><topic>Cardiovascular disease</topic><topic>Case-Control Studies</topic><topic>Complications and side effects</topic><topic>Congenital diseases</topic><topic>CREST = variant of sclerodermacharacterized by calcinosis, Raynaud's phenomenon, esophageal motilitydisorders, sclerodactyly, and telangiectasia</topic><topic>Drug therapy</topic><topic>epoprostenol</topic><topic>Epoprostenol - therapeutic use</topic><topic>Female</topic><topic>Heart</topic><topic>Hemodynamics</topic><topic>Humans</topic><topic>Hypertension, Pulmonary - drug therapy</topic><topic>Hypertension, Pulmonary - etiology</topic><topic>Hypertension, Pulmonary - physiopathology</topic><topic>Intubation</topic><topic>Lupus</topic><topic>Lupus Erythematosus, Systemic - complications</topic><topic>Medical sciences</topic><topic>mPAP = mean pulmonaryartery pressure</topic><topic>Nitric oxide</topic><topic>PAP = pulmonary artery pressure</topic><topic>Patients</topic><topic>Pharmacology. Drug treatments</topic><topic>PPH = primarypulmonary hypertension</topic><topic>Pulmonary arteries</topic><topic>Pulmonary hypertension</topic><topic>Pulmonary Wedge Pressure - drug effects</topic><topic>PVR = pulmonary vascular resistance</topic><topic>Respiratory system</topic><topic>Scleroderma</topic><topic>SLE = systemic lupus erythematosus</topic><topic>Systemic lupus erythematosus</topic><topic>Treatment Outcome</topic><topic>Vascular Resistance - drug effects</topic><topic>Vasodilation - drug effects</topic><topic>Veins & arteries</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Robbins, Ivan M.</creatorcontrib><creatorcontrib>Gaine, Sean P.</creatorcontrib><creatorcontrib>Schilz, Robert</creatorcontrib><creatorcontrib>Tapson, Victor F.</creatorcontrib><creatorcontrib>Rubin, Lewis J.</creatorcontrib><creatorcontrib>Loyd, James E.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Proquest Nursing & Allied Health Source</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>Consumer Health Database (Alumni Edition)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Consumer Health Database</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Nursing & Allied Health Premium</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>Chest</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Robbins, Ivan M.</au><au>Gaine, Sean P.</au><au>Schilz, Robert</au><au>Tapson, Victor F.</au><au>Rubin, Lewis J.</au><au>Loyd, James E.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Epoprostenol for Treatment of Pulmonary Hypertension in Patients With Systemic Lupus Erythematosus</atitle><jtitle>Chest</jtitle><addtitle>Chest</addtitle><date>2000-01</date><risdate>2000</risdate><volume>117</volume><issue>1</issue><spage>14</spage><epage>18</epage><pages>14-18</pages><issn>0012-3692</issn><eissn>1931-3543</eissn><coden>CHETBF</coden><abstract>Pulmonary hypertension with pathologicalchanges similar to those observed in primary pulmonary hypertensionoccurs in patients with systemic lupus erythematosus (SLE). Theefficacy of chronic epoprostenol therapy in SLE has not been welldescribed. The objective of this paper is to describe our experiencewith long-term epoprostenol therapy in patients with pulmonaryhypertension associated with SLE.
Case seriesof six patients with SLE and associated pulmonary hypertensionreceiving chronic treatment with epoprostenol.
All 6 patients had severe pulmonary hypertension.Mean pulmonary artery pressure (mPAP) was 57 ± 9 mm Hg (mean±SD), and pulmonary vascular resistance was 14 ± 7 units beforebeginning therapy with epoprostenol. In 4 patients who underwent repeathemodynamic evaluation (9 to 16 months after starting epoprostenol), mean pulmonary artery pressure decreased by 38 ± 21% andpulmonary vascular resistance by 58 ± 12%. Clinically, allpatients improved from New York Heart Association class III or IV toclass I or II. Doses of epoprostenol ranged from 4 to 46 ng/kg/min, andthe longest duration of therapy has been 2.5 years. Side effects fromepoprostenol have not differed from those seen in patients with primarypulmonary hypertension, and except for one patient, there has been noexacerbation of SLE.
Epoprostenol waseffective for the treatment of pulmonary hypertension in this smallgroup of patients with SLE. Further evaluation of epoprostenol therapyfor patients with SLE and other diseases associated with pulmonaryhypertension is warranted.</abstract><cop>Northbrook, IL</cop><pub>Elsevier Inc</pub><pmid>10631192</pmid><doi>10.1378/chest.117.1.14</doi><tpages>5</tpages></addata></record> |
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subjects | Adult Antihypertensive Agents - therapeutic use Biological and medical sciences Cardiac Catheterization Cardiovascular disease Case-Control Studies Complications and side effects Congenital diseases CREST = variant of sclerodermacharacterized by calcinosis, Raynaud's phenomenon, esophageal motilitydisorders, sclerodactyly, and telangiectasia Drug therapy epoprostenol Epoprostenol - therapeutic use Female Heart Hemodynamics Humans Hypertension, Pulmonary - drug therapy Hypertension, Pulmonary - etiology Hypertension, Pulmonary - physiopathology Intubation Lupus Lupus Erythematosus, Systemic - complications Medical sciences mPAP = mean pulmonaryartery pressure Nitric oxide PAP = pulmonary artery pressure Patients Pharmacology. Drug treatments PPH = primarypulmonary hypertension Pulmonary arteries Pulmonary hypertension Pulmonary Wedge Pressure - drug effects PVR = pulmonary vascular resistance Respiratory system Scleroderma SLE = systemic lupus erythematosus Systemic lupus erythematosus Treatment Outcome Vascular Resistance - drug effects Vasodilation - drug effects Veins & arteries |
title | Epoprostenol for Treatment of Pulmonary Hypertension in Patients With Systemic Lupus Erythematosus |
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