The Experts below are selected from a list of 258 Experts worldwide ranked by ideXlab platform
Marc Humbert - One of the best experts on this subject based on the ideXlab platform.
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Immunosuppressive Therapy in lupus and mixed connective tissue disease associated pulmonary arterial hypertension a retrospective analysis of twenty three cases
Arthritis & Rheumatism, 2008Co-Authors: Xavier Jais, Olivier Sitbon, Gerald Simonneau, David Launay, Azzedine Yaici, Jerome Le Pavec, C Tcherakian, Marc HumbertAbstract:Objective To describe the response to first-line Immunosuppressive Therapy with or without pulmonary vasodilators in pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MCTD). Methods Twenty-three consecutive patients with SLE- or MCTD-associated PAH treated with first-line Immunosuppressive Therapy either alone (n = 16) or in combination with pulmonary vasodilators (n = 7) were evaluated according to clinical and hemodynamic criteria before and after Immunosuppressive Therapy. Responders were defined as patients in New York Heart Association (NYHA) functional class I or II with hemodynamic improvement after the last pulse of cyclophosphamide. Results Among the 16 patients treated with first-line Immunosuppressive Therapy alone, 8 (50%) were responders. These patients had a significantly improved NYHA functional class, 6-minute walking distance, and mean pulmonary artery pressure. Patients in NYHA functional class I or II and/or a cardiac index >3.1 liters/minute/m2 at baseline were more likely to benefit from Immunosuppressive Therapy. Six of the 8 nonresponders subsequently improved with pulmonary vasodilators. Among the 7 patients who were initially treated with Immunosuppressive Therapy and pulmonary vasodilators, 4 (57.1%) were responders. Conclusion PAH associated with SLE or MCTD may respond to a treatment combining cyclophosphamide and glucocorticoids. Patients who could benefit from this Immunosuppressive Therapy could be those who have less severe disease at baseline. For patients with more severe disease, pulmonary vasodilators should be started, possibly in combination with immunosuppressants. In any case, clinical and hemodynamic evaluations are mandatory to monitor the response and adapt the treatment. These retrospective and uncontrolled data need to be confirmed by randomized controlled trials.
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Immunosuppressive Therapy in connective tissue diseases associated pulmonary arterial hypertension
Chest, 2006Co-Authors: Olivier Sanchez, Olivier Sitbon, Xavier Jais, Gerald Simonneau, Marc HumbertAbstract: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/m 2 , 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 Conclusion PAH associated with SLE or MCTD might respond to a treatment combining glucocorticosteroids and cyclophosphamide.
Xavier Jais - One of the best experts on this subject based on the ideXlab platform.
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Immunosuppressive Therapy in lupus and mixed connective tissue disease associated pulmonary arterial hypertension a retrospective analysis of twenty three cases
Arthritis & Rheumatism, 2008Co-Authors: Xavier Jais, Olivier Sitbon, Gerald Simonneau, David Launay, Azzedine Yaici, Jerome Le Pavec, C Tcherakian, Marc HumbertAbstract:Objective To describe the response to first-line Immunosuppressive Therapy with or without pulmonary vasodilators in pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MCTD). Methods Twenty-three consecutive patients with SLE- or MCTD-associated PAH treated with first-line Immunosuppressive Therapy either alone (n = 16) or in combination with pulmonary vasodilators (n = 7) were evaluated according to clinical and hemodynamic criteria before and after Immunosuppressive Therapy. Responders were defined as patients in New York Heart Association (NYHA) functional class I or II with hemodynamic improvement after the last pulse of cyclophosphamide. Results Among the 16 patients treated with first-line Immunosuppressive Therapy alone, 8 (50%) were responders. These patients had a significantly improved NYHA functional class, 6-minute walking distance, and mean pulmonary artery pressure. Patients in NYHA functional class I or II and/or a cardiac index >3.1 liters/minute/m2 at baseline were more likely to benefit from Immunosuppressive Therapy. Six of the 8 nonresponders subsequently improved with pulmonary vasodilators. Among the 7 patients who were initially treated with Immunosuppressive Therapy and pulmonary vasodilators, 4 (57.1%) were responders. Conclusion PAH associated with SLE or MCTD may respond to a treatment combining cyclophosphamide and glucocorticoids. Patients who could benefit from this Immunosuppressive Therapy could be those who have less severe disease at baseline. For patients with more severe disease, pulmonary vasodilators should be started, possibly in combination with immunosuppressants. In any case, clinical and hemodynamic evaluations are mandatory to monitor the response and adapt the treatment. These retrospective and uncontrolled data need to be confirmed by randomized controlled trials.
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Immunosuppressive Therapy in connective tissue diseases associated pulmonary arterial hypertension
Chest, 2006Co-Authors: Olivier Sanchez, Olivier Sitbon, Xavier Jais, Gerald Simonneau, Marc HumbertAbstract: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/m 2 , 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 Conclusion PAH associated with SLE or MCTD might respond to a treatment combining glucocorticosteroids and cyclophosphamide.
Gerald Simonneau - One of the best experts on this subject based on the ideXlab platform.
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Immunosuppressive Therapy in lupus and mixed connective tissue disease associated pulmonary arterial hypertension a retrospective analysis of twenty three cases
Arthritis & Rheumatism, 2008Co-Authors: Xavier Jais, Olivier Sitbon, Gerald Simonneau, David Launay, Azzedine Yaici, Jerome Le Pavec, C Tcherakian, Marc HumbertAbstract:Objective To describe the response to first-line Immunosuppressive Therapy with or without pulmonary vasodilators in pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MCTD). Methods Twenty-three consecutive patients with SLE- or MCTD-associated PAH treated with first-line Immunosuppressive Therapy either alone (n = 16) or in combination with pulmonary vasodilators (n = 7) were evaluated according to clinical and hemodynamic criteria before and after Immunosuppressive Therapy. Responders were defined as patients in New York Heart Association (NYHA) functional class I or II with hemodynamic improvement after the last pulse of cyclophosphamide. Results Among the 16 patients treated with first-line Immunosuppressive Therapy alone, 8 (50%) were responders. These patients had a significantly improved NYHA functional class, 6-minute walking distance, and mean pulmonary artery pressure. Patients in NYHA functional class I or II and/or a cardiac index >3.1 liters/minute/m2 at baseline were more likely to benefit from Immunosuppressive Therapy. Six of the 8 nonresponders subsequently improved with pulmonary vasodilators. Among the 7 patients who were initially treated with Immunosuppressive Therapy and pulmonary vasodilators, 4 (57.1%) were responders. Conclusion PAH associated with SLE or MCTD may respond to a treatment combining cyclophosphamide and glucocorticoids. Patients who could benefit from this Immunosuppressive Therapy could be those who have less severe disease at baseline. For patients with more severe disease, pulmonary vasodilators should be started, possibly in combination with immunosuppressants. In any case, clinical and hemodynamic evaluations are mandatory to monitor the response and adapt the treatment. These retrospective and uncontrolled data need to be confirmed by randomized controlled trials.
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Immunosuppressive Therapy in connective tissue diseases associated pulmonary arterial hypertension
Chest, 2006Co-Authors: Olivier Sanchez, Olivier Sitbon, Xavier Jais, Gerald Simonneau, Marc HumbertAbstract: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/m 2 , 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 Conclusion PAH associated with SLE or MCTD might respond to a treatment combining glucocorticosteroids and cyclophosphamide.
Olivier Sitbon - One of the best experts on this subject based on the ideXlab platform.
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Immunosuppressive Therapy in lupus and mixed connective tissue disease associated pulmonary arterial hypertension a retrospective analysis of twenty three cases
Arthritis & Rheumatism, 2008Co-Authors: Xavier Jais, Olivier Sitbon, Gerald Simonneau, David Launay, Azzedine Yaici, Jerome Le Pavec, C Tcherakian, Marc HumbertAbstract:Objective To describe the response to first-line Immunosuppressive Therapy with or without pulmonary vasodilators in pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MCTD). Methods Twenty-three consecutive patients with SLE- or MCTD-associated PAH treated with first-line Immunosuppressive Therapy either alone (n = 16) or in combination with pulmonary vasodilators (n = 7) were evaluated according to clinical and hemodynamic criteria before and after Immunosuppressive Therapy. Responders were defined as patients in New York Heart Association (NYHA) functional class I or II with hemodynamic improvement after the last pulse of cyclophosphamide. Results Among the 16 patients treated with first-line Immunosuppressive Therapy alone, 8 (50%) were responders. These patients had a significantly improved NYHA functional class, 6-minute walking distance, and mean pulmonary artery pressure. Patients in NYHA functional class I or II and/or a cardiac index >3.1 liters/minute/m2 at baseline were more likely to benefit from Immunosuppressive Therapy. Six of the 8 nonresponders subsequently improved with pulmonary vasodilators. Among the 7 patients who were initially treated with Immunosuppressive Therapy and pulmonary vasodilators, 4 (57.1%) were responders. Conclusion PAH associated with SLE or MCTD may respond to a treatment combining cyclophosphamide and glucocorticoids. Patients who could benefit from this Immunosuppressive Therapy could be those who have less severe disease at baseline. For patients with more severe disease, pulmonary vasodilators should be started, possibly in combination with immunosuppressants. In any case, clinical and hemodynamic evaluations are mandatory to monitor the response and adapt the treatment. These retrospective and uncontrolled data need to be confirmed by randomized controlled trials.
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Immunosuppressive Therapy in connective tissue diseases associated pulmonary arterial hypertension
Chest, 2006Co-Authors: Olivier Sanchez, Olivier Sitbon, Xavier Jais, Gerald Simonneau, Marc HumbertAbstract: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/m 2 , 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 Conclusion PAH associated with SLE or MCTD might respond to a treatment combining glucocorticosteroids and cyclophosphamide.
David Launay - One of the best experts on this subject based on the ideXlab platform.
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Immunosuppressive Therapy in lupus and mixed connective tissue disease associated pulmonary arterial hypertension a retrospective analysis of twenty three cases
Arthritis & Rheumatism, 2008Co-Authors: Xavier Jais, Olivier Sitbon, Gerald Simonneau, David Launay, Azzedine Yaici, Jerome Le Pavec, C Tcherakian, Marc HumbertAbstract:Objective To describe the response to first-line Immunosuppressive Therapy with or without pulmonary vasodilators in pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MCTD). Methods Twenty-three consecutive patients with SLE- or MCTD-associated PAH treated with first-line Immunosuppressive Therapy either alone (n = 16) or in combination with pulmonary vasodilators (n = 7) were evaluated according to clinical and hemodynamic criteria before and after Immunosuppressive Therapy. Responders were defined as patients in New York Heart Association (NYHA) functional class I or II with hemodynamic improvement after the last pulse of cyclophosphamide. Results Among the 16 patients treated with first-line Immunosuppressive Therapy alone, 8 (50%) were responders. These patients had a significantly improved NYHA functional class, 6-minute walking distance, and mean pulmonary artery pressure. Patients in NYHA functional class I or II and/or a cardiac index >3.1 liters/minute/m2 at baseline were more likely to benefit from Immunosuppressive Therapy. Six of the 8 nonresponders subsequently improved with pulmonary vasodilators. Among the 7 patients who were initially treated with Immunosuppressive Therapy and pulmonary vasodilators, 4 (57.1%) were responders. Conclusion PAH associated with SLE or MCTD may respond to a treatment combining cyclophosphamide and glucocorticoids. Patients who could benefit from this Immunosuppressive Therapy could be those who have less severe disease at baseline. For patients with more severe disease, pulmonary vasodilators should be started, possibly in combination with immunosuppressants. In any case, clinical and hemodynamic evaluations are mandatory to monitor the response and adapt the treatment. These retrospective and uncontrolled data need to be confirmed by randomized controlled trials.
