An Update on the Evaluation and Management of Pulmonary Hypertension in Scleroderma

Coghlan, J. Gerry; Schrieber, Benjamin

doi:10.1007/s11926-011-0226-2

Cited by 5 publications

(4 citation statements)

References 55 publications

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“…36 Since sites differ in rates of use of PAH-specific therapies, including combination therapy, randomisation will be stratified according to site, with the effect of various PAH therapies subsequently adjusted for in multiple regression analysis.…”

Section: Methods and Analysismentioning

confidence: 99%

Multicentre randomised placebo-controlled trial of oral anticoagulation with apixaban in systemic sclerosis-related pulmonary arterial hypertension: the SPHInX study protocol

et al. 2016

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IntroductionSystemic sclerosis (SSc) is a severe and costly multiorgan autoimmune connective tissue disease characterised by vasculopathy and fibrosis. One of the major causes of SSc-related death is pulmonary arterial hypertension (PAH), which develops in 12–15% of patients with SSc and accounts for 30–40% of deaths. In situ thrombosis in the small calibre peripheral pulmonary vessels resulting from endothelial dysfunction and an imbalance of anticoagulant and prothrombotic mediators has been implicated in the complex pathophysiology of SSc-related PAH (SSc-PAH), with international clinical guidelines recommending the use of anticoagulants for some types of PAH, such as idiopathic PAH. However, anticoagulation has not become part of standard clinical care for patients with SSc-PAH as only observational evidence exists to support its use. Therefore, we present the rationale and methodology of a phase III randomised controlled trial (RCT) to evaluate the efficacy, safety and cost-effectiveness of anticoagulation in SSc-PAH.Methods and analysisThis Australian multicentre RCT will compare 2.5 mg apixaban with placebo, in parallel treatment groups randomised in a 1:1 ratio, both administered twice daily for 3 years as adjunct therapy to stable oral PAH therapy. The composite primary outcome measure will be the time to death or clinical worsening of PAH. Secondary outcomes will include functional capacity, health-related quality of life measures and adverse events. A cost-effectiveness analysis of anticoagulation versus placebo will also be undertaken.Ethics and disseminationEthical approval for this RCT has been granted by the Human Research Ethics Committees of all participating centres. An independent data safety monitoring board will review safety and tolerability data for the duration of the trial. The findings of this RCT are to be published in open access journals.Trial registration numberACTRN12614000418673, Pre-results.

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Section: Methods and Analysismentioning

confidence: 99%

Multicentre randomised placebo-controlled trial of oral anticoagulation with apixaban in systemic sclerosis-related pulmonary arterial hypertension: the SPHInX study protocol

et al. 2016

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“…Aggressive immunosuppression considered of some benefit in other autoimmune diseaserelated pulmonary hypertension such as SLE-and MCTDrelated pulmonary hypertension has not been found to be effective in SS-PAH although the role of new immunomodulatory agents is under investigation [158,159] . One of the most challenging areas in managing SS-PAH patients is the timely diagnosis of the disease and the selection of those patients with diffuse cutaneous SS who develop 'disproportionally' high mean pulmonary artery pressure values, implying beyond a causative relation to the existing ILD an intrinsic vascular process that could potentially respond to PAH specific treatment [160,161] . Lung and/or heart transplantation remains the last option in carefully selected SS-PAH patients [162] .…”

Section: Pulmonary Arterial Hypertensionmentioning

confidence: 99%

Investigation of Lung Involvement in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2015

Respiration

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Lung involvement in connective tissue disorders (CTDs) may present as pleomorphic since any lung compartment may be involved such as airways, exocrine secretory and alveolar epithelia, interstitial lung structure, pulmonary vasculature and pleura as well as, in specific disorders, several tissues of the thoracoabdominal ventilator pump. Any combination of the above anatomic structures may be involved concomitantly although some specific combinations may include a determinant of rheumatic disorders. The diagnosis of a specific CTD requires the fulfilment of clearly defined clinical and laboratory criteria including in most cases positivity for autoantibodies, mostly specific serologic combinations. In this setting, serologic investigation targets mainly, although not exclusively, the detection of antinuclear antibodies. A specific serologic positivity or a combination of autoantibodies constitutes not only a diagnostic criterion for a specific CTD, but may also characterize the pattern of respiratory manifestation in a determinant rheumatic disorder. Therefore, the investigation of lung involvement in CTDs requires adequate skills in the ambit of a multidisciplinary approach and an extended spectrum of diagnostic tools and modalities able to detect both early clinical clues and serologic conversion as well as any pathophysiologic alteration that regards the complexity of respiratory functional status. Although many patients with CTDs suffer from a ‘vicious' combination of lung involvement, lung drug toxicity and infections related to the above two as well as to the ‘mater' disease, for space reasons this review will focus on the established lung manifestations that regard the 7 major CTDs.

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“…[22][23][24][25] Anticoagulation is therefore not currently considered 'standard of care' in CTD-PAH.…”

Section: Current Treatment Guidelines Reflect a State Of Clinical Equmentioning

confidence: 99%

“…Whereas the American Heart Association and the American College of Chest Physicians support the use of anticoagulation with a grade 'B' recommendation (a moderate recommendation), based on observational studies that indicate a survival benefit in anticoagulated iPAH patients, the recommendation for anticoagulation in CTD-PAH is considerably weaker and based on expert opinion only. [22][23][24][25] Anticoagulation is therefore not currently considered 'standard of care' in CTD-PAH.…”

Section: Current Treatment Guidelines Reflect a State Of Clinical Equmentioning

confidence: 99%

Should patients with systemic sclerosis‐related pulmonary arterial hypertension be anticoagulated?

Nikpour

Stevens

Proudman

et al. 2013

Internal Medicine Journal

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Pulmonary arterial hypertension (PAH) is a major cause of mortality in scleroderma and despite 'advanced' therapies confers a median survival of less than 5 years. Anticoagulation in systemic sclerosis-related PAH (SSc-PAH) is currently one of the most contentious issues in the management of patients with connective tissue disease. While some studies have shown a survival benefit with warfarin therapy in this disease, others have not. Accordingly, a state of clinical equipoise exists in relation to anticoagulation in SSc-PAH. With an over fivefold reduction in mortality demonstrated in some observational studies, the issue of anticoagulation in SSc-PAH demands resolution through a well-designed randomised controlled trial.

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An Update on the Evaluation and Management of Pulmonary Hypertension in Scleroderma

Cited by 5 publications

References 55 publications

Multicentre randomised placebo-controlled trial of oral anticoagulation with apixaban in systemic sclerosis-related pulmonary arterial hypertension: the SPHInX study protocol

Multicentre randomised placebo-controlled trial of oral anticoagulation with apixaban in systemic sclerosis-related pulmonary arterial hypertension: the SPHInX study protocol

Investigation of Lung Involvement in Connective Tissue Disorders

Should patients with systemic sclerosis‐related pulmonary arterial hypertension be anticoagulated?

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