| Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. | |
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MedLine Citation:
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PMID: 16840400 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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STUDY OBJECTIVE: Immune and inflammatory mechanisms could play a significant role in pulmonary arterial hypertension (PAH) genesis or progression, especially in patients with connective tissue diseases. Immunosuppressive therapy should be better evaluated in this setting.Study design: Monocentric retrospective study. PATIENTS: We reviewed the clinical and hemodynamic effects of immunosuppressants administered as first-line monotherapy to 28 consecutive patients with connective tissue disease-associated PAH. INTERVENTIONS: All patients received a monthly IV bolus of cyclophosphamide, 600 mg/m2, for at least 3 months, and 22 of 28 patients received systemic glucocorticosteroids. Responders to immunosuppressive therapy were defined as patients who remained in New York Heart Association (NYHA) functional class I or II with sustained hemodynamic improvement after at least 1 year of immunosuppressive therapy without addition of prostanoids, phosphodiesterase type 5 inhibitors, or endothelin receptor antagonists. RESULTS: Eight of 28 patients (systemic lupus erythematosus [SLE], n = 5; mixed connective tissue disease [MCTD], n = 3) [29%] were responders. These patients had a significantly improved 6-min walking distance (available in five patients) and a significant improvement in hemodynamic function. No patients with systemic sclerosis responded, while 5 of 12 patients with SLE and 3 of 8 patients with MCTD did respond. Survival analysis indicated that responders had a better survival than nonresponders. Patients with a lower baseline NYHA functional class and better baseline pulmonary hemodynamics (p < 0.05) were more likely to benefit from immunosuppressive therapy. CONCLUSION: PAH associated with SLE or MCTD might respond to a treatment combining glucocorticosteroids and cyclophosphamide. |
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Authors:
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Olivier Sanchez; Olivier Sitbon; Xavier Jaïs; Gérald Simonneau; Marc Humbert |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Chest Volume: 130 ISSN: 0012-3692 ISO Abbreviation: Chest Publication Date: 2006 Jul |
Date Detail:
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Created Date: 2006-07-14 Completed Date: 2006-08-10 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0231335 Medline TA: Chest Country: United States |
Other Details:
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Languages: eng Pagination: 182-9 Citation Subset: AIM; IM |
Affiliation:
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Centre National de Référence de l'Hypertension Artérielle Pulmonaire, UPRES EA2705, Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Assistance Publique Hôpitaux de Paris, Université Paris-Sud, Clamart, France. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Blood Pressure / drug effects Connective Tissue Diseases / classification, complications, drug therapy* Cyclophosphamide / therapeutic use* Exercise Test / methods Female Glucocorticoids / therapeutic use* Humans Hypertension, Pulmonary / drug therapy, etiology*, physiopathology Immunosuppressive Agents / therapeutic use* Male Middle Aged Retrospective Studies Severity of Illness Index |
| Chemical | |
Reg. No./Substance:
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0/Glucocorticoids; 0/Immunosuppressive Agents; 50-18-0/Cyclophosphamide |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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