Ambrisentan For Sarcoidosis Associated Pulmonary Hypertension (SAPH)

Judson, Marc A.; Ford, H. James; Donohue, James F.; Aris, John P.; Craft, Nicole; Burt, Sharikia; Highland, Kristin B.

doi:10.1164/ajrccm-conference.2010.181.1_meetingabstracts.a2368

Cited by 43 publications

(58 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Improvement in 6MWD has been found with some patients after therapy [29]. However, changes in 6MWD were not demonstrated in other intervention studies [48][49][50].…”

Section: Pulmonary Functionmentioning

confidence: 98%

Established and experimental medical therapy of pulmonary sarcoidosis

et al. 2013

View full text Add to dashboard Cite

The treatment options for pulmonary sarcoidosis have increased over the past 10 years. As new treatments have been introduced, the best way to assess and compare treatments remains unknown. The goal of this review is to discuss the standard treatments for pulmonary sarcoidosis, including glucocorticoids, and cytotoxic agents, such as methotrexate, azathioprine and leflunomide, and compare them to the newer biological agents, such as infliximab and adalimumab. We also discuss some novel treatments which are currently being evaluated. To compare these different regimens, we look at the measures used to assess response. These include pulmonary function, chest imaging, steroid sparing potential and, more recently, improvements in quality of life measures. While there is, as yet, no standard assessment for response, there is a growing consensus that response to treatment may include improvement of one or more of the following: forced vital capacity, chest imaging and steroid sparing. Several drugs used for pulmonary sarcoidosis have demonstrated improvement in one or more of these measures.

show abstract

“…Improvement in 6MWD has been found with some patients after therapy [29]. However, changes in 6MWD were not demonstrated in other intervention studies [48][49][50].…”

Section: Pulmonary Functionmentioning

confidence: 98%

Established and experimental medical therapy of pulmonary sarcoidosis

et al. 2013

View full text Add to dashboard Cite

show abstract

“…By contrast, bosentan was found to be entirely nonefficacious in fibrotic IIP-PH [57] but may have beneficial effects in some sarcoidosis-PH patients, especially in patients with PH and limited ILD, although the fragmentary nature of current data must be emphasised [84][85][86][87]. Ambrisentan appears to be poorly tolerated in sarcoidosis-PH [88]. In an open-label, uncontrolled ILD-PH trial, riociguat, a stimulator of the soluble guanylate cyclase, was shown to increase cardiac output, decrease PVR and improve exercise capacity as judged by the effect in the 6MWT [89].…”

Section: Treatmentmentioning

confidence: 99%

Comorbidities in interstitial lung diseases

2017

View full text Add to dashboard Cite

Fibrosing lung disorders include a large number of diseases with diverse behaviour. Patients can die because of the progression of their illness, remain stable or even improve after appropriate treatment has been instituted. Comorbidities, such as acute and chronic infection, gastro-oesophageal reflux, pulmonary hypertension, lung cancer, cardiovascular diseases, and obstructive sleep apnoea, can pre-exist or develop at any time during the course of the disease and, if unidentified and untreated, may impair quality of life, impact upon the respiratory status of the patients, and ultimately lead to disease progression and death. Therefore, early identification and accurate treatment of comorbidities is essential.

show abstract

“…There has been one randomised, placebo-controlled study of ambrisentan, a selective ET receptor A blocker, in 17 patients with sarcoidosisrelated PH. Unfortunately this was a negative study that has only been reported in abstract form thus far [55]. A large prospective randomised 16-week study of bosentan in this patient population is currently underway, with an anticipated completion date of December 2012 [56].…”

Section: The Et Pathwaymentioning

confidence: 99%

Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation

Shlobin¹,

Nathan²

2012

Eur Respir J

View full text Add to dashboard Cite

Sarcoidosis is not only a multisystem, but also a multinational disease that is prevalent throughout the world, including Europe, the USA and Japan. Lung involvement in sarcoidosis is seemingly invariable, with up to 95% of patients manifesting some form of pulmonary disease during the course of their lifetime. The natural history of sarcoidosis in the lung is quite variable and spans the spectrum from spontaneous resolution to advanced fibrocystic disease in ,5% of cases. Advanced sarcoidosis will be the subject of this review with a special focus on pulmonary hypertension and lung transplantation as a last-resort treatment option for some patients with end-stage disease.

show abstract

Ambrisentan For Sarcoidosis Associated Pulmonary Hypertension (SAPH)

Cited by 43 publications

References 0 publications

Established and experimental medical therapy of pulmonary sarcoidosis

Established and experimental medical therapy of pulmonary sarcoidosis

Comorbidities in interstitial lung diseases

Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation

Contact Info

Product

Resources

About