Long-term administration of beraprost, an oral prostacyclin analogue, improves pulmonary diffusion capacity in patients with systemic sclerosis

Matsukawa, Yoshihisa; Saito, O.; Aoki, Masaru; Abe, Mitsunari; Nishinarita, Susumu; Shibata, Shigeki; Horie, Takashi; Naruse, Satoshi; Hiranuma, Maya

doi:10.1054/plef.2002.0380

Cited by 10 publications

(4 citation statements)

References 28 publications

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“…Generally, SSc-PAH patients have similar, but less robust responses to pulmonary hypertension therapies as patients with IPAH [53,56–78,79 ▪ ]. Randomized control trials of oral and intravenous prostacyclins, ambrisentan, and combination therapy with ambrisentan and tadadafil have demonstrated improvements in hemodynamics, diffusion limitation, exercise capacity, WHO functional class, time to clinical worsening, and number of clinical worsening events for SSc-PAH patients [53,56,59,65,70,79 ▪ ]. Other studies have yielded similar results with additional improvements in NT-proBNP levels, patient-reported symptoms, and health-related quality of life by studying SSc-PAH as a sub-group of all PAH [57,58,60–62,64,80,81 ▪▪ ,82,83 ▪▪ ].…”

Section: Treatment and Outcomesmentioning

confidence: 94%

“…A number of studies have evaluated the efficacy of each class of PAH medication as monotherapy or in combination therapy for patients with SSc-PAH (Table 2). Generally, SSc-PAH patients have similar, but less robust responses to pulmonary hypertension therapies as patients with IPAH [53,56–78,79 ▪ ]. Randomized control trials of oral and intravenous prostacyclins, ambrisentan, and combination therapy with ambrisentan and tadadafil have demonstrated improvements in hemodynamics, diffusion limitation, exercise capacity, WHO functional class, time to clinical worsening, and number of clinical worsening events for SSc-PAH patients [53,56,59,65,70,79 ▪ ].…”

Section: Treatment and Outcomesmentioning

confidence: 99%

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Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension?

Khan,

Mathai

2023

Current Opinion in Pulmonary Medicine

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Purpose of review Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc), which confers significant morbidity and mortality. The current therapies and treatment strategies for SSc-associated PAH (SSc-PAH) are informed by those used to treat patients with idiopathic PAH (IPAH). There are, however, important differences between these two diseases that impact diagnosis, treatment, and outcomes. Recent findings Both SSc-PAH and IPAH are incompletely understood with ongoing research into the underlying cellular biology that characterize and differentiate the two diseases. Additional research seeks to improve identification among SSc patients in order to diagnose patients earlier in the course of their disease. Novel therapies specifically for SSc-PAH such as rituximab and dimethyl fumarate are under investigation. Summary Although patients with SSc-PAH and IPAH present with similar symptoms, there are significant differences between these two forms of PAH that warrant further investigation and characterization of optimal detection strategies, treatment algorithms, and outcomes assessment.

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Section: Treatment and Outcomesmentioning

confidence: 94%

Section: Treatment and Outcomesmentioning

confidence: 99%

Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension?

Khan,

Mathai

2023

Current Opinion in Pulmonary Medicine

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“…Seventeen patients, with SSc and DLCO of less than 95%, received beraprost sodium for at least 12 months. DLCO levels in patients with SSc improved after the administration of beraprost sodium, probably due to the decrease in pulmonary vascular resistance accompanied by increased cardiac output (Matsukawa et al 2002).…”

Section: Raynaud's Phenomena/pulmonary Hypertensionmentioning

confidence: 96%

New Therapeutic Strategies for Systemic Sclerosis—a Critical Analysis of the Literature

Zandman‐Goddard

Tweezer-Zaks

Shoenfeld

2005

Journal of Immunology Research

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Systemic sclerosis (SSc) is a multi-system disease characterized by skin fibrosis and visceral disease. Therapy is organ and pathogenesis targeted. In this review, we describe novel strategies in the treatment of SSc. Utilizing the MEDLINE and the COCHRANE REGISTRY, we identified open trials, controlled trials, for treatment of SSc from 1999 to April 2005. We used the terms scleroderma, systemic sclerosis, Raynaud's phenomenon, pulmonary hypertension, methotrexate, cyclosporin, tacrolimus, relaxin, low-dose penicillamine, IVIg, calcium channel blockers, losartan, prazocin, iloprost, N-acetylcysteine, bosentan, cyclophosphamide, lung transplantation, ACE inhibitors, anti-thymocyte globulin, and stem cell transplantation. Anecdotal reports were omitted. Methotrexate, cyclosporin, tacrolimus, relaxin, low-dose penicillamine, and IVIg may be beneficial in improving the skin tightness in SSc. Calcium channel blockers, the angiotensin II receptor type 1 antagonist losartan, prazocin, the prostacyclin analogue iloprost, N-acetylcysteine and the dual endothelin-receptor antagonist bosentan may be beneficial for Raynaud's phenomenon. Epoprostenol and bosentan are approved for therapy of pulmonary hypertension (PAH). Other options under investigation include intravenous or aerolized iloprost. Cyclophosphamide (CYC) pulse therapy is effective in suppressing active alveolitis. Stem cell and lung transplantation is a viable option for carefully selected patients. Renal crisis can be effectively managed when hypertension is aggressively controlled with angiotensin converting enzyme (ACE) inhibitors. Patients should continue taking ACE inhibitors even after beginning dialysis in hope of discontinuing dialysis. Anti-thymocyte globulin and mycophenolate mofetil appear safe in SSc. The improvement in skin score and the apparent stability of systemic disease during the treatment period suggest that controlled studies of these agents are justified. Stem cell transplantation is under investigation for severe disease. Novel therapies are currently being tested in the treatment of SSc and have the potential of modifying the disease process and overall clinical outcome. The evaluation of these studies is still a difficult process.

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“…Bei 107 Patienten, die mit Beraprost über 6-12 Monate behandelt wurden, ergab sich ein Trend zur Verhütung des Auftretens akraler Ulzerationen [49]. Eine offene Studie an 17 Patienten mit einer progressiven Skerodermie [50] zeigte, dass sich bei 10 von 12 Patienten, die diese Studie beendeten, die Diffusionskapazität der Lunge verbessert hatte. Diese Studie weist ebenso wie die Arbeit von Scorza et al [43] auf eine mögliche Wirkung von Prostazyklinen auf die Beteiligung innerer Organe.…”

Section: Introductionunclassified

Medikament�se vasoaktive Therapien von Mikrozirkulationsst�rungen bei rheumatischen Erkrankungen

Riemekasten

Schulze‐Koops

2005

Z. Rheumatol.

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Long-term administration of beraprost, an oral prostacyclin analogue, improves pulmonary diffusion capacity in patients with systemic sclerosis

Cited by 10 publications

References 28 publications

Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension?

Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension?

New Therapeutic Strategies for Systemic Sclerosis—a Critical Analysis of the Literature

Medikament�se vasoaktive Therapien von Mikrozirkulationsst�rungen bei rheumatischen Erkrankungen

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