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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Veröffentlicht in:Chest 2000-01, Vol.117 (1), p.14-18
Hauptverfasser: Robbins, Ivan M., Gaine, Sean P., Schilz, Robert, Tapson, Victor F., Rubin, Lewis J., Loyd, James E.
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container_end_page 18
container_issue 1
container_start_page 14
container_title Chest
container_volume 117
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.
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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. 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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. 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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 &amp; 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 &amp; Allied Health Source</collection><collection>Health &amp; 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 &amp; Medical Complete (Alumni)</collection><collection>Nursing &amp; Allied Health Database (Alumni Edition)</collection><collection>Consumer Health Database</collection><collection>Health &amp; Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Nursing &amp; 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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