Bosentan for Sarcoidosis-Associated Pulmonary Hypertension

Baughman, Robert P.; Culver, Daniel A.; Cordova, Francis; Padilla, María L.; Gibson, Kevin F.; Lower, Elyse E.; Engel, Peter J.

doi:10.1378/chest.13-1766

Cited by 128 publications

(23 citation statements)

References 32 publications

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“…Endothelin receptor antagonists have been more extensively investigated, particularly bosentan. One double-blind, placebo-controlled trial investigated 35 PH patients with all different sarcoidosis stages, presenting with New York Heart Association class II or III symptoms, low PAWP and stable on immunosuppressive therapy for at least three months [ 26 ]. Bosentan improved the mean PAP and pulmonary vascular resistance.…”

Section: Managementmentioning

confidence: 99%

Pulmonary hypertension complicating pulmonary sarcoidosis

et al. 2016

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Pulmonary hypertension (PH) is a severe complication of sarcoidosis, with an unknown prevalence. The aetiology is multifactorial, and the exact mechanism of PH in the individual patient is often difficult to establish. The diagnostic work-up and treatment of PH in sarcoidosis is complex, and should therefore be determined by a multidisciplinary expert team in a specialised centre. It is still a major challenge to identify sarcoidosis patients at risk for developing PH. There is no validated algorithm when to refer a patient suspected for PH, and PH analysis itself is difficult. Until present, there is no established therapy for PH in sarcoidosis. Besides optimal treatment for sarcoidosis, case series evaluating new therapeutic options involving PH-targeted therapy are arising for a subgroup of patients. This review summarises the current knowledge regarding the aetiology, diagnosis and possible treatment options for PH in sarcoidosis.

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Section: Managementmentioning

confidence: 99%

Pulmonary hypertension complicating pulmonary sarcoidosis

et al. 2016

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“…Case reports and case series have demonstrated a benefit in a subgroup of patients with sarcoidosis associated pulmonary hypertension who have received phosphodiesterase inhibitors,230243245 endothelial receptor antagonists,235243245246 and intravenous and inhaled prostacylins 247248. One randomized placebo controlled trial (involving 30 evaluable patients) of bosentan for sarcoidosis associated pulmonary hypertension showed that bosentan but not placebo significantly reduced mean pulmonary arterial pressure and decreased pulmonary vascular resistance 249. There was no statistically significant change in 6 minute walk distance for either group.…”

Section: Management Of Difficult Clinical Issuesmentioning

confidence: 99%

New advances in the management of pulmonary sarcoidosis

Culver

Judson

2019

BMJ

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Sarcoidosis is a highly variable granulomatous multisystem syndrome. It affects individuals in the prime years of life; both the frequency and severity of sarcoidosis are greater in economically disadvantaged populations. The diagnosis, assessment, and management of pulmonary sarcoidosis have evolved as new technologies and therapies have been adopted. Transbronchial needle aspiration guided by endobronchial ultrasound has replaced mediastinoscopy in many centers. Advanced imaging modalities, such as fluorodeoxyglucose positron emission tomography scanning, and the widespread availability of magnetic resonance imaging have led to more sensitive assessment of organ involvement and disease activity. Although several new insights about the pathogenesis of sarcoidosis exist, no new therapies have been specifically developed for use in the disease. The current or proposed use of immunosuppressive medications for sarcoidosis has been extrapolated from other disease states; various novel pathways are currently under investigation as therapeutic targets. Coupled with the growing recognition of corticosteroid toxicities for managing sarcoidosis, the use of corticosteroid sparing anti-sarcoidosis medications is likely to increase. Besides treatment of granulomatous inflammation, recognition and management of the non-granulomatous complications of pulmonary sarcoidosis are needed for optimal outcomes in patients with advanced disease.

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“…However, no significant change of the 6MWD was observed in an open label prospective study assessing the efficacy of inhaled iloprost [113]. However, only one randomised clinical trial was performed in this group of patient showing a significant improvement in pulmonary haemodynamics, although no significant change in 6MWD was observed [114].…”

Section: Treatmentmentioning

confidence: 99%

Pulmonary hypertension and chronic lung disease: where are we headed?

et al. 2019

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Pulmonary hypertension related to chronic lung disease, mainly represented by COPD and idiopathic pulmonary fibrosis, is associated with a worse outcome when compared with patients only affected by parenchymal lung disease. At present, no therapies are available to reverse or slow down the pathological process of this condition and most of the clinical trials conducted to date have had no clinically significant impact. Nevertheless, the importance of chronic lung diseases is always more widely recognised and, along with its increasing incidence, associated pulmonary hypertension is also expected to be growing in frequency and as a health burden worldwide. Therefore, it is desirable to develop useful and reliable tools to obtain an early diagnosis and to monitor and follow-up this condition, while new insights in the therapeutic approach are explored.

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Bosentan for Sarcoidosis-Associated Pulmonary Hypertension

Cited by 128 publications

References 32 publications

Pulmonary hypertension complicating pulmonary sarcoidosis

Pulmonary hypertension complicating pulmonary sarcoidosis

New advances in the management of pulmonary sarcoidosis

Pulmonary hypertension and chronic lung disease: where are we headed?

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